Canadian Journal of Physiology and Pharmacology

Riboflavin and pyrroloquinoline generate carbon monoxide in the presence of tissue microsomes or recombinant human cytochrome P-450 oxidoreductase (CPR); implications for possible roles in gasotransmission.

Journal: Canadian Journal of Physiology and Pharmacology

Manuscript ID cjpp-2019-0376.R1

Manuscript Type: Article

Date Submitted by the 19-Oct-2019 Author:

Complete List of Authors: Vukomanovic, Dragic; Queens University Jia, Zongchao; Queens University Nakatsu, Kanji;Draft Queen's University, Pharmacology & Toxicology Smith, Graeme; Queen's University, Anatomy and Cell Biology; Kingston General Hospital, Obstetrics and Gynecology Ozolinš, Terence; Queen's University, Biomedical and Molecular Sciences

Is the invited manuscript for consideration in a Special Not applicable (regular submission) Issue:

• Riboflavin, • Pyrroloquinoline quinone, • Carbon monoxide, • Keyword: Cytochrome P450 oxidoreductase, Human placenta microsomes

https://mc06.manuscriptcentral.com/cjpp-pubs Page 1 of 28 Canadian Journal of Physiology and Pharmacology

1

1 Riboflavin and pyrroloquinoline quinone generate carbon monoxide in the presence of

2 tissue microsomes or recombinant human cytochrome P-450 oxidoreductase (CPR);

3 implications for possible roles in gasotransmission.

4

5 Dragic Vukomanovica,b, Zongchao Jiaa, Kanji Nakatsua, and Graeme N. Smitha,b Terence R.S.

6 Ozolinša

7 a. Department of Biomedical and Molecular Sciences, Queen’s University, Kingston, Ontario,

8 Canada, K7L 3N6

9 b. Department of Obstetrics and Gynaecology, Kingston General Hospital, Kingston, Ontario,

10 Canada, K7L 3N6

11 Draft

12 CORRESPONDING AUTHOR:

13 Dr. Terence R.S. Ozolinš, Department of Biomedical & Molecular Sciences,

14 Queen’s University, Kingston, ON, Canada

15 K7L 3N6

16 Tel 613-533-3306 Facsimile 613-533-6412

17 [email protected]

18

19

20 There are no declarations of interest for any of the authors.

21

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 2 of 28

2

22 ABSTRACT

23 Carbon monoxide (CO), an endogenously produced gasotransmitter, regulates inflammation and

24 vascular tone, suggesting delivery of CO may be therapeutically useful for pathologies like

25 preeclampsia where CO insufficiency are implicated. Our strategy is to identify chemicals that

26 increase the activity of endogenous CO producing , including cytochrome P-450

27 oxidoreductase (CPR). Realizing both riboflavin and pyrroloquinoline quinone (PQQ) are

28 relatively non-toxic, even at high doses, and they share chemical properties with toxic CO

29 activators we previously identified, our goal was to determine whether riboflavin or PQQ could

30 stimulate CO production. Riboflavin and PQQ were incubated in sealed vessels with rat and

31 human tissue extracts and CO generation was measured with headspace-gas chromatography.

32 Riboflavin and PQQ increased CO productionDraft ~ 60% in rat spleen microsomes. In rat brain

33 microsomes, riboflavin and PQQ increased respective CO production ~four-fold and two-fold,

34 compared to baseline. CO production by human placenta microsomes increased four-fold with

35 riboflavin and five-fold with PQQ. In the presence of recombinant human CPR, CO production

36 was three-fold greater with PQQ than riboflavin. These observations demonstrate for the first

37 time that riboflavin and PQQ facilitate tissue-specific CO production with significant

38 contributions from CPR. We propose a novel biochemical role for these nutrients in

39 gastransmission.

40 Keywords:

41  Riboflavin

42  Pyrroloquinoline quinone

43  Carbon monoxide

44  Human placenta microsomes

https://mc06.manuscriptcentral.com/cjpp-pubs Page 3 of 28 Canadian Journal of Physiology and Pharmacology

3

45  Cytochrome P-450 oxidoreductase

46

47 Novelty Bullets:

48

49  Riboflavin or PQQ stimulated CO generation in a tissue-specific manner

50  Recombinant human cytochrome P-450 reductase (CPR) generates CO in the presence of

51 riboflavin or PQQ

52  CO production is a novel biochemical function for these nutrients

53  Riboflavin or PQQ may increase CO production in conditions of CO insufficiency such as

54 preeclampsia Draft 55

56

57

58

59

60

61

62

63

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 4 of 28

4

64 INTRODUCTION

65 Carbon monoxide (CO) is an important gasotransmitter critical in certain signalling

66 pathways related to vascular tone and the production of inflammatory mediators (reviewed in

67 Motterlini & Foresti 2017). Thus, the actions of CO may have clinical applications in many

68 diseases associated with vascular dysfunction and/or inflammation, including preeclampsia

69 (Phipps et al. 2019). The development of therapeutic CO delivery is an area of active research

70 and includes exogenous CO delivery methods and the development of CO-releasing molecules

71 (CORMs (Ismailova et al. 2018; Kim & Choi 2018). Another strategy, one that we favour, is to

72 generate CO in situ by activating endogenous CO-producing enzymes.

73

74 The primary endogenous sourceDraft of CO is from oxygenase during heme degradation

75 (Motterlini & Foresti 2017). There are two primary forms of mammalian heme oxygenases

76 (HO). HO-2 is predominantly expressed in brain and testes, and although HO-1 is highly

77 inducible following oxidative stress, the spleen is a rich source of its constitutive expression

78 (Vukomanovic et al. 2014). Recently, we demonstrated CO is also produced by cytochrome P-

79 450 oxidoreductase (CPR) (Vukomanovic et al. 2017), indicating at least three significant

80 enzymatic sources of CO. The CO-producing enzymes HO-1, HO-2 and CPR reside in the

81 endoplasmic reticulum (Riddick et al. 2013; Motterlini and Foresti 2017), and when cells are

82 disrupted, the endoplasmic reticulum forms heterologous vesicles, which after differential

83 centrifugation yield a highly enriched source of endoplasmic reticulum enzymes, i.e.

84 microsomes. We use microsomes as an in vitro screening tool to understand the important

85 structure activity relationships (SAR) necessary to stimulate CO production (Vukomanovic et al.

86 2011; Vukomanovic et al. 2014). Briefly, we came to realize that the capacity of a compound to

https://mc06.manuscriptcentral.com/cjpp-pubs Page 5 of 28 Canadian Journal of Physiology and Pharmacology

5

87 enhance CO production was not associated with a traditional pharmacophore, but instead

88 appeared related to redox activities inherent to the structure; a potent prototypic CO generator is

89 menadione, a synthetic analogue of K (K3). Using menadione, a subsequent series of

90 studies identified a novel enzymatic source of CO production. Briefly, the use of specific HO-2

91 inhibitors with rat brain microsomes, and recombinant forms of HO-2 and CPR allowed us to

92 conclude that the menadione-mediated CO production that we had previously noted in rat brain

93 extracts was primarily the result of CPR, not HO-2 (Vukomanovic et al. 2017).

94 We have identified a number of synthetic isoalloxazine derivatives as potent CO

95 producers (unpublished data). Isoalloxazine is a tricyclic heterocycle from which flavins are

96 derived, and we reasoned that a biochemical source of flavin may provide an acceptable safety

97 profile. Riboflavin is the most abundantDraft biochemical source of flavin and although the riboflavin

98 recommended daily allowance (RDA) is 1.3 milligrams daily for men and 1.1 mg for women, it

99 may be consumed orally as high as 400 mg/day to treat migraine headaches with few adverse

100 side effects (Powers 2003; Thakur et al. 2016). Thus, there is compelling rationale to test

101 whether riboflavin stimulates CO generation.

102

103 Naphthoquinones, are also potent CO generators (Vukomanovic et al. 2011), but the dose

104 of menadione required to increase CO levels in vivo was toxic (Odozor et al. 2018).

105 Recognizing the need to identify less toxic naturally occurring naphthoquinones, we investigated

106 vitamin K1 (phylloquinone) and K2 (menaquinone; MK-4), but disappointingly, neither was

107 effective at producing CO in spleen or brain microsomes (Vukomanovic et al. 2014). This

108 prompted a search for alternate compounds with a quinone or quinone-like nucleus, and evidence

109 of clinical safety. Pyrroloquinoline quinone (PQQ; 4,5-dioxo-4,5-dihydro-1H-pyrrolo[2,3-

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 6 of 28

6

110 f]-2,7,9-tricarboxylic acid), an aromatic tricyclic o-quinone, is an important nutrient,

111 found in a variety of foods, although its role as a vitamin remains controversial (reviewed in

112 Misra et al. 2012; Akagawa, et al. 2016; Ames 2018). PQQ has a number of proposed health

113 benefits including reduction of pro-inflammatory markers (Harris et al. 2013), a

114 pathophysiological factor in preeclampsia. Together, these observations, and its limited toxicity

115 when taken orally (Akagawa et al., 2016), made PQQ a promising candidate for CO production.

116

117 The goal of our study was to determine whether riboflavin and PQQ promote CO

118 production in microsomes from several tissue sources, including human placenta, the organ in

119 which CO insufficiency has been implicated in the etiology of preeclampsia (Levytska et al.

120 2013; Venditti and Smith, 2014). Draft

121

122

123 MATERIAL AND METHODS

124

125 Preparation of Riboflavin and PQQ

126 A 10.0 mM stock solution of riboflavin 5’-phosphate sodium salt hydrate (Sigma-Aldrich Ltd,

127 Toronto, Canada, catalogue #R7774) was prepared in double distilled water. A 10.0 mM stock

128 solution of PQQ (ChemScene LLC, Monmouth Junction NJ, USA, catalogue # CS-W009276)

129 was prepared in DMSO.

130

131 Preparation of rat spleen and brain microsomal fractions

https://mc06.manuscriptcentral.com/cjpp-pubs Page 7 of 28 Canadian Journal of Physiology and Pharmacology

7

132 Male rats were obtained from Charles River Ltd (St-Constant, Canada) and the organs were

133 harvested under approved protocols and in accordance with Queen’s University Animal Care

134 Committee and the Canadian Council on Animal Care’s Guide to the Care and Use of

135 Experimental Animals. The microsomal fractions of rat spleen (Braggins et al. 1986) and rat

136 brain (Trakshel et al 1988), were prepared by differential centrifugation of tissue homogenate as

137 previously described (Appleton et al. 1999; Kinobi et al. 2006). Protein concentration of the

138 microsomal fraction was determined by a modification of the Biuret method (Cook et al. 1995).

139 The microsomal fraction was resuspended in 100 mM phosphate buffer/20% (v/v), glycerol

140 solution containing 10 mM EDTA and stored at –80 °C until used (Kinobi et al. 2006).

141

142 Collection and perfusion of human placentasDraft

143 Term human placentas (>37 weeks) from uncomplicated pregnancies were obtained following

144 elective Caesarean section at the Kingston General Hospital (Kingston, Ontario), under the

145 approval of the Health Sciences Research Ethics Board at Queen’s University (OBGY-290-16).

146 Maternal identifiers were not collected in order to maintain confidentiality and anonymity.

147 Following removal from the uterus, the umbilical cord remained clamped to maintain blood

148 pressure and the placenta was placed on ice for transport to the laboratory.

149

150 After removal of the amnion on the placental surface, a peripheral chorionic plate artery

151 was cannulated and continuously perfused using a peristaltic pump (Gilson Minipuls 3, Gilson

152 Inc. USA, Middleton WI) with ice cold Krebs’ buffer (Ahmed et al. 2005) at the rate of 20

153 mL/min. Perfusate and blood were allowed to drain from the paired vein during perfusion of the

154 cotyledon. When the cotyledon on the fetal side became pale, perfusion was initiated on the

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 8 of 28

8

155 maternal side by inserting a catheter into the cotyledon until the entire cotyledon appeared

156 bloodless. The perfused cotyledon was then excised, and the rest of the placenta disposed.

157

158 Preparation of human placenta microsomal fractions

159 The microsomal fractions from perfused human placenta tissue were prepared by differential

160 centrifugation of tissue homogenate as previously described (Vukomanovic et al. 2010). Protein

161 concentration of the microsomal fraction was determined using a modification of the Biuret

162 method, as we described previously (Cook et al. 1995). The microsomal fraction was

163 resuspended in a 100 mM phosphate buffer/20% (v/v), glycerol solution containing 10 mM

164 EDTA and stored at -80 °C.

165 Draft

166 Preparation of recombinant human CPR (rh-CPR)

167 The hCPR-pET22b vector encoding full-length human CPR was a generous gift from Dr. Paul

168 Ortiz de Montellano (University of San Francisco). Expression and purification were based on

169 protocols previously described (Dierks et al. 1998). In brief, the plasmid was transformed into

170 BL21 (DE3) pLysS cells that were cultured in Terrific Broth (TB) supplemented with trace

171 elements and riboflavin in the presence of ampicillin and chloramphenicol. Induction of

172 expression was initiated with 0.4 M isopropyl-β-D-thiogalactopyranoside (IPTG). When the

173 absorbance (A) at 600 nm had reached ~0.4 the cells were allowed to grow further overnight at

174 30°C. Spheroplasts were generated from the harvested cells prior to lysis by sonication. Lysates

175 were centrifuged at 10,000 r/min (Ti45 rotor) and the supernatant further centrifuged at 35,000

176 r/min (Ti45 rotor) to pellet the membrane fraction which was subsequently stored at –80°C for

177 future purification.

https://mc06.manuscriptcentral.com/cjpp-pubs Page 9 of 28 Canadian Journal of Physiology and Pharmacology

9

178

179 Membrane pellets were thawed, then solubilized in 20 mM potassium phosphate (pH

180 7.4), 20% (v/v) glycerol, 0.2% Triton X-100, 0.1 mM EDTA, 1 mM PMSF, 0.2% sodium cholate

181 with one Roche EDTA-free protease cocktail tablet. After clarification, the supernatant was

182 purified over a 2′5′-ADP-Sepharose column, which had been equilibrated with Buffer A (20 mM

183 potassium phosphate (pH 7.4), 20% glycerol, 0.1 mM EDTA, 0.2% sodium cholate). The column

184 was washed with Buffer A, followed by washes in the presence of 250 mM NaCl, and in the

185 presence of 2 mM adenosine. Elution of the rh-CPR protein was performed with 5 mM 2’AMP.

186 Purified protein was dialyzed against 40 mM potassium phosphate, 20% glycerol overnight at

187 4°C, concentrated, and quantified by Bradford assay using bovine serum albumin (Sigma-

188 Aldrich USA Inc.) as a standard. ProteinDraft was aliquoted, flash-frozen in liquid nitrogen and stored

189 at –80°C.

190

191 Enzymatic assay for CO generation

192 CO generation by human placenta microsomal fractions was determined with a gas

193 chromatographic method using headspace-gas analysis as described by (Vukomanovic et al.

194 2014). The incubation conditions had been previously optimized with respect to concentrations

195 of reaction components and the duration of incubation (Vukomanovic et al. 2017). Briefly, a

196 reaction mixture (150 μL) containing 100 mM phosphate buffer (pH 7.4), 50 μM

197 methemalbumin and (1 mg microsomal protein or 10 μM recombinant protein), was pre-

198 incubated with an riboflavin or PQQ concentration ranging from 0.01 to 100 μM, for 10 min at

199 37 °C in a shaking water-bath and then each vial’s head space was purged with CO-free air for

200 15s. The reaction was initiated by adding adenine dinucleotide phosphate

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 10 of 28

10

201 (NADPH; Sigma-Aldrich Ltd, Toronto, Canada) to a final concentration of 1.0 mM, and was

202 continued for 15 min at 37 °C. The reaction was stopped by instantly freezing the reaction

203 mixture on dry ice. The generated CO in the head-space was quantified using a specially

204 designed gas-chromatograph for CO analysis (ta 3000R Process Gas Analyzer; Ametek Inc.

205 USA, Newark, DE) which is sensitive and selective for CO by virtue of headspace sampling, size

206 exclusion gas chromatography and mercuric oxide reduction detection (Vreman and Stevenson,

207 1988). Peak heights and areas were used to correlate detector response between standards and

208 unknowns in the linear range. CO production by samples and blanks, analyzed in triplicate, was

209 calculated and HO activity is presented as the net difference between total and blank reactions

210 expressed as pmoles of CO. Controls in which methemalbumin, NADPH or enzymes were

211 omitted have been reported previously (VukomanovicDraft et al. 2011).

212

213 Western Blot Analysis

214 The rh-CPR preparation (5 μg of protein) was run on a 10% SDS-PAGE gel, and stained with

215 Coomasie Brilliant Blue. A molecular weight ladder used to confirm protein identity (Precision

216 Plus ProteinTM Standards; Biorad Ltd., Mississauga Canada, cat#161-0373).

217

218 Statistical analysis

219 Data were expressed as the mean ± SD and analyzed by one-way ANOVA, followed by a

220 Newman-Keuls multiple comparison test for a significant F statistic (P < 0.05) (Prism 7;

221 GraphPad Software, San Diego CA). A P value less than 0.05 was considered to be significant.

222

223 RESULTS

https://mc06.manuscriptcentral.com/cjpp-pubs Page 11 of 28 Canadian Journal of Physiology and Pharmacology

11

224 All microsomal preparations showed significant baseline CO production of approximately 70

225 pmol (Figs.1-3).

226

227 Riboflavin and PQQ are activators of CO production in spleen

228 At concentrations below 25 µM, riboflavin was a more potent activator of CO production than

229 PQQ. For example, riboflavin stimulated increased CO production at concentrations as low as

230 100 nM, whereas the threshold for PQQ-mediated CO production was 25 µM (Fig 1). At 100

231 µM, the highest concentration used, riboflavin and PQQ increased CO production to 169±10 and

232 157±15 respectively, reflecting an approximately two-fold over baseline. Rat spleen microsomes

233 contain CPR and HO-1, but comparatively little HO-2, implicating the former two enzymes at

234 the catalytic source of CO. Draft

235

236 PQQ is superior to riboflavin at activating CO production in brain

237 The baseline for CO generated by rat brain microsomes was approximately 75 pmol (Fig. 2). The

238 addition of PQQ over the range of 0.01 to 1 µM did not increase CO production significantly.

239 After the addition of 25 µM of PQQ the production of CO increased to 148±12 pmol, and the

240 presence of 100 µM PQQ, increased CO to 303±70 pmol (Fig. 2). The CO generation curve for

241 riboflavin was similar to that of PQQ except at the highest concentration (100 µM) riboflavin

242 increased CO production to 175±9 pmol.

243

244 Riboflavin and PQQ stimulate CO production in human placenta

245 Having demonstrated CO production by riboflavin and PQQ in rodent tissue, we tested whether

246 human tissue contained similar biochemical machinery by preparing placental microsomes from

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 12 of 28

12

247 healthy infant deliveries. The baseline for CO generated by human placenta microsomes was

248 approximately 70 pmol, and addition of 1 µM PQQ increased CO production to 112±18 pmol

249 (Fig. 3). The addition of 10 and 25 µM PQQ increased CO production to 210±35 and 299±48,

250 respectively. The CO production curve of riboflavin was shifted approximately one order of

251 magnitude to the right with 10 µM producing no detectable increase in CO and the addition of 25

252 µM riboflavin only yielding 129±24 pmol CO production. At 100 µM PQQ and riboflavin

253 increased CO generation to 370±27 and 280±62 pmol, reflecting respectively, an approximate

254 five- and four-fold change from 10 nM.

255

256 PQQ produces significantly more CO than riboflavin in the presence of rh-CPR

257 To determine the extent to which CPR mayDraft contribute to CO production in the human placenta,

258 experiments were conducted where riboflavin and PQQ were incubated in the presence of

259 recombinant human CPR (rh-CPR) and the necessary substrates and cofactors; methemalbumin

260 and NADPH, respectively. Unlike the microsomal preparations, baseline levels of CO

261 production were nearly undetectable in the reconstituted system (Fig. 4A). In the presence of 1

262 µM PQQ, 9±3pmol of CO was generated while 10 µM PQQ increased this to 75±17 pmol CO.

263 The further escalation to 100 µM increased CO production to 159 ± 17 pmol. Relative to the

264 PQQ CO generation curve, the riboflavin CO generation curve was shifted approximately two

265 orders of magnitude to the right. Here, the first significant increase in CO generation occurred at

266 100 µM riboflavin (47±15 pmol CO; Fig 4A). The rh-CPR containing preparation used in Fig. 4

267 was run on 10% SDS-PAGE (Fig. 4B). A very dense over-saturated band corresponding to rh-

268 CPR was identified at approximately 75 kDa with several fainter minor contaminants identified

269 at higher and lower molecular weights.

https://mc06.manuscriptcentral.com/cjpp-pubs Page 13 of 28 Canadian Journal of Physiology and Pharmacology

13

270

271 DISCUSSION

272

273 There were two critical observations in this study. First, both riboflavin and PQQ stimulated

274 significant CO production in the presence of microsomes prepared from rat spleen, rat brain or

275 human placenta. The second critical observation is that riboflavin and PQQ also stimulated the

276 generation of CO in the presence of purified rh-CPR.

277 The preparation containing rh-CPR had as its major component an oversaturated band at

278 approximately 75 kDa corresponding closely to the previously reported molecular weight of

279 approximately 76.5 kDa (Uniprot.org). Although faint bands of contaminating proteins were

280 present it is unlikely they contributed toDraft CO generation. First, by comparison rh-CHR was

281 present at exponentially greater quantity than all the contaminants combined. Second, although

282 HO have been reported in a number of bacteria, only one has been reported in E. Coli, the

283 species from which the BL21 (DE3) pLysS cells, used to grow the expression vector, are

284 derived. In E. Coli, the HO is called ChuS (Maharshak et al. 2015) and is reported to run at

285 approximately 39 kDa (Uniprot.org). There is a faint shadow corresponding to this approximate

286 molecular weight (Fig 4B) but is many orders of magnitude less abundant than rh-CPR

287 suggesting it does not contribute to CO production.

288 In view of CO’s role as an important second messenger gas (reviewed in Motterlini and

289 Foresti 2017), these observations suggest that in addition to their well-described functions as

290 enzymatic co-factors (reviewed in Ames 2018; Powers 2003; Thakur et al. 2017), riboflavin and

291 PQQ may have hitherto unreported roles in the regulation of CO-dependent physiological

292 signalling. All microsomal preparations in the current study showed a significant baseline level

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 14 of 28

14

293 of CO production approximating 70 pmol. In the presence of menadione, we demonstrated

294 baseline CO arises from contributions by HO, whereas the asymptotic component of the CO

295 generation curve is primarily the result of CPR (Vukomanovic et al. 2017). Therefore, we

296 ascribe the CO production observed at higher concentrations of riboflavin and PQQ to also be

297 predominantly CPR. It has long been held that CO production in mammalian systems is the

298 domain of heme-oxygenase enzymes (reviewed in Maines 1997), but these current observations

299 further underscore our recent studies showing the importance of CPR in physiological CO

300 production (Vukomanovic et al. 2017). CO generation occurred in persistent tissues such as

301 spleen and brain, as well as the placenta which exists transiently, suggesting that significant CO

302 generation may occur in any tissue containing CPR.

303 Draft

304 Our interest in identifying agents that stimulate CO generation in vivo stems from a

305 growing body of work implicating CO insufficiency in the etoiology of preeclampsia

306 (Bainbridge et al. 2005; Levytska et al. 2013; George et al. 2014; Ramma and Ahmed 2014;

307 Venditti and Smith 2014). Indeed, a mouse model of preeclampsia was used to demonstrate, that

308 direct inhalation of CO augments uterine artery blood flow and uteroplacental vascular growth

309 (Vendetti et al. 2013) and protected against associated maternal organ damage (Venditti et al.

310 2014). The present study demonstrating that riboflavin stimulates CO generation in human

311 placenta microsomes may provide a mechanistic link to the observation that riboflavin deficiency

312 is significant risk factor for preeclampsia (Wacker et al. 2000). A subsequent riboflavin

313 intervention study was unable to demonstrate a clinical benefit for the treatment of preeclampsia

314 (Neugebauer et al. 2006); however, we speculate the “low dose” 15 mg/day used in the

315 aforementioned study was insufficient. For instance, multiple migraine intervention trials have

https://mc06.manuscriptcentral.com/cjpp-pubs Page 15 of 28 Canadian Journal of Physiology and Pharmacology

15

316 used “high dose” 400 mg/day riboflavin to effectively reduce the incidence and severity of

317 migraines (reviewed in Thakur et al. 2017). Our current studies demonstrating significant CO

318 production from brain microsomes, lead us to propose that the therapeutic efficacy of riboflavin

319 against migraines may be, in part, CO-dependent; recalling that CO is an important mediator of

320 inflammation and vascular tone (Motterlini and Foresti 2017), both of which are believed to be

321 dysregulated in migraine (Conti et al. 2019). A role for targeted riboflavin supplementation in

322 the prevention of hypertension has also been identified in patients with a specific defect in

323 methylenetetrahydrofolatereductase (MTHFR) (reviewed in McNulty et al. 2017). These

324 randomized trials showed that riboflavin has a genotype-specific role in lowering blood pressure

325 by an average of 6-13 mmHg independent of the effect of antihypertensive drugs. The

326 mechanism of action that has been proposedDraft is that riboflavin supplementation in patients with

327 MTHFR677TT genotype might lead to improved endothelial function through increased

328 bioavailability of nitric oxide via increases in both 5-methyltetrahydrofolate and

329 . Our current findings suggest that CO generation may also contribute to a

330 portion of riboflavin’s antihypertensive effects. We are unaware of studies linking PQQ with

331 preeclampsia or migraines, but in a small clinical crossover study, supplementation with PQQ

332 significantly reduced circulating markers of inflammation (Harris et al. 2013), an effect also

333 ascribed to CO (reviewed in Motterlini and Foresti 2017). Taken together, our in vitro findings

334 showing riboflavin and PQQ activation of CO production are consistent with some of the in vivo

335 clinical observations ascribed to dietary supplementation with these two nutrients.

336 In the current study, in vitro CO production from human and rat microsomes was

337 detectable when riboflavin and PQQ approached the μM range, whereas the reported in vivo

338 tissue concentrations of riboflavin and PQQ are nM (respectively, Bosch et al. 2011; Kumazawa

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 16 of 28

16

339 et al. 1992). Despite this concentration discrepancy our prior studies suggest riboflavin and PQQ

340 will promote CO production in vivo. For instance, in rat brain microsomes menadione increased

341 CO production at μM and not nM concentrations (Vukomanovic et al. 2011), yet when added to

342 the drinking water of mice, menadione significantly increased maternal CO concentrations

343 (Odozur et al. 2018). Although plasma menadione concentrations were not measured in the

344 aforementioned study, it is unlikely that plasma menadione concentrations exceeded the nM

345 range. First, taste aversion reduces water consumption when menadione is added to the drinking

346 water (Odozur et al. 2018), and low μM concentrations have only been reported when

347 menadione was administered i.v. at toxic doses as an adjunct to cancer chemotherapy (Lim et al.

348 2005).

349 The present study showed that 100Draft µM riboflavin or PQQ increased CO production in

350 human placenta microsomes approximately three- or four- fold, over baseline respectively. This

351 compares favourably with the six-fold increase in CO production in the presence of 100 µM

352 menadione (unpublished data). The manner in which riboflavin and PQQ enhance CO

353 production remains to be elucidated; however, we speculate their mechanism of action may be

354 similar to that of menadione, because these nutrients share a number of electrochemical and

355 structural similarities with the analogue. Previously, we demonstrated that the ability

356 of menadione to promote CO production from rh-CPR was reliant upon the generation of

357 reactive oxygen species in the form of superoxide radicals and hydrogen peroxide; addition of

358 superoxide dismutase and catalase significantly reduced CO production (Vukomanovic et al.

359 2017). The ability of riboflavin and PQQ to act as cofactors free radical formation has been

360 previously reported (respectively, Juárez et al. 2008; Akagawa et al. 2016).

https://mc06.manuscriptcentral.com/cjpp-pubs Page 17 of 28 Canadian Journal of Physiology and Pharmacology

17

361 There were tissue-specific differences in CO production noted with both nutrients. The

362 elucidation of the cause of the tissue-specific effects would require further experimentation, but

363 we expect the differences to be, in part, the result of differences in the relative ratios of CPR,

364 HO-1 and HO-2 enzyme activities. For example, we previously demonstrated that menadione

365 stimulates robust CO production in rat brain microsomes, a constitutively rich source of HO-2;

366 however, in spleen an abundant source of HO-1, menadione fails to stimulate CO production

367 (Vukomanovic et al. 2014). In the present study, the addition of riboflavin and PQQ

368 significantly increased CO production in rat spleen microsomes, but the response was greater in

369 brain microsomes suggesting HO-2 and CPR activities are higher in this tissue. Although subtle,

370 riboflavin was the more effective promoter of CO production in the spleen whereas in rat brain

371 and human placenta PQQ was dominant.Draft What is more, in placenta microsomes and with

372 preparations only containing rh-CPR, there was a markedly similar rightward shift of the

373 riboflavin CO production curve relative to that of PQQ. We interpret the shared rightward shift

374 to mean, that with respect to riboflavin and PQQ, CPR rather than HO is the dominant source of

375 CO production in the human placenta. The importance of CPR may have profound clinical

376 implications. For instance, placental HO-1 expression and exhaled CO levels are both lower in

377 women with preeclampsia, consistent with a role for HO-1 as an important source of CO during

378 pregnancy (Levytska et al. 2013). Lower HO activity in cases of preeclampsia presents a

379 profound challenge, recalling that our therapeutic strategy is reliant using a compromised

380 endogenous biochemical process (HO-1) to produce CO. The observation, that CPR is likely the

381 dominant CO generation activity in the human placenta leads us to suggest that riboflavin or

382 PQQ may be used to activate CPR-based CO production to compensate for the HO insufficiency.

383 Additionally, the extraordinary increase of CO production by RB or PQQ becomes even more

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 18 of 28

18

384 significant in the light of Zorn and Wells (2010) pointing out that even a modest enzymatic

385 activity enhancement of 10% is therapeutically useful. Thus, the several-fold increase in

386 placental CO production in the presence of RB or PQQ may have therapeutic potential even if

387 the endogenous process used to produce CO is compromised.

388

389 In conclusion, we have described previously that CO production occurs in chorionic villi

390 of human placenta (Ahmed et al. 2005), and the current studies demonstrate for the first time that

391 riboflavin and PQQ increase CO production in human placenta microsomes. Thus, we propose

392 that both nutrients may have novel biochemical roles in gasotransmission. A critical role for CPR

393 in the production of CO was also demonstrated, and the further study of this novel pathway

394 would be warranted because its up- or down-regulationDraft by nutrients may have therapeutic

395 applications.

396

397

398 ACKNOWLEDGEMENTS

399 We thank the nurses and surgeons at Kingston General Hospital for access to placentas. The

400 assistance of Laura van Staalduinen in the conduct of the Western blot analysis is gratefully

401 acknowledged.

402

403 FUNDING SOURCES

404 This research was funded by the Canadian Institutes of Health Research Grant CIP-150737

405 ABBREVIATIONS

406 CO: carbon monoxide

https://mc06.manuscriptcentral.com/cjpp-pubs Page 19 of 28 Canadian Journal of Physiology and Pharmacology

19

407 CPR: cytochrome p450 reductase

408 HO: heme oxygenase

409 NADPH: nicotinamide adenine dinucleotide phosphate

410 PQQ: pyrroloqinoline-quinone

411 RB: riboflavin

412

413 REFERENCES

414 415 Ahmed, H., McLaughlin, B.E., Song, J., Marks, G.S., Brien, J.F., Nakatsu, K. 2005. The source 416 of endogenous carbon monoxide formation in human placental chorionic villi. Cell. Mol. Biol. 417 51:447-51. PMID:16309566. 418 419 Akagawa, M., Nakano, M., Ikemoto, K. 2016. Recent progress in studies on the health benefits 420 of pyrroloquinoline quinone. Biosci. Biotech.Draft Biochem. 80:13–22. 421 http://doi:10.1080/09168451.2015.1062715. PMID:26168402. 422 423 Ames, B.N., 2018. Prolonging healthy aging: Longevity and proteins. Proc. Natl. Acad. 424 Sci. U S A. 115:10836-10844. http://doi:10.1073/pnas.1809045115. PMID:30322941. 425 426 Appleton, S.D., Chretien, M.L., McLaughlin, B.E., Vreman, H.J., Stevenson, D.K., Brien, J.F., 427 Nakatsu, K., Maurice, D.H., Marks, G.S. 1999. Selective inhibition of heme oxygenase, without 428 inhibition of nitric oxide synthase or soluble guanylyl cyclase, by metalloporphyrins at low 429 concentrations. Drug Metab. Dispos. 27:1214-9. PMID:10497150. 430 431 Bainbridge, S.A., Sidle, E.H., Smith, G.N. 2005. Direct placental effects of cigarette smoke 432 protect women from pre-eclampsia: the specific roles of carbon monoxide and 433 systems in the placenta. Med. Hypotheses. 64:17-27. http://doi:10.1016/j.mehy.2004.06.019. 434 PMID:15533604. 435 436 Bosch, A.M., Abeling, N.G., Ijlst, L., Knoester, H., van der Pol, W.L., Stroomer, A.E., Wanders, 437 R.J., Visser, G., Wijburg, F.A., Duran, M., Waterham, H.R. 2011. Brown-Vialetto-Van Laere 438 and Fazio Londe syndrome is associated with a riboflavin transporter defect mimicking mild 439 MADD: a new inborn error of metabolism with potential treatment. J. Inherit. Metab. Dis. 440 34:159-64. http://doi:10.1007/s10545-010-9242-z. PMID:21110228. 441 442 Braggins, P.E., Trakshel, G.M., Kutty, R.K., Maines, M.D. 1986. Characterization of two heme 443 oxygenase isoforms in rat spleen: comparison with the hematin-induced and constitutive 444 isoforms of the liver. Biochem. Biophys. Res. Commun. 141:528-33. http://doi:10.1016/s0006- 445 291x(86)80205-4. PMID:3099789.

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 20 of 28

20

446 447 Conti, P., D'Ovidio, C., Conti, C., Gallenga, C.E., Lauritano, D., Caraffa, A., Kritas, S.K., 448 Ronconi, G. 2019. Progression in migraine: Role of mast cells and pro-inflammatory and anti- 449 inflammatory cytokines. Eur. J. Pharmacol. 844:87-94. http://doi:10.1016/j.ejphar.2018.12.004. 450 PMID:30529470. 451 452 Cook, M.N., Nakatsu, K., Marks, G.S., McLaughlin, B.E., Vreman, H.J., Stevenson, D.K., Brien, 453 J.F. 1995. Heme oxygenase activity in the adult rat aorta and liver as measured by carbon 454 monoxide formation. Can. J. Physiol. Pharmacol. 73:515-8. PMID:7671194. 455 456 Dierks, E.A., Davis, S.C., Ortiz de Montellano, P.R. 1998. Glu-320 and Asp-323 are 457 determinants of the CYP4A1 hydroxylation regio- specificity and resistance to inactivation by 1- 458 aminobenzotriazole. Biochemistry 37:1839-47. http://doi:10.1021/bi972458s. PMID:9485309. 459 460 George, E.M., Warrington, J.P., Spradley, F.T., Palei, A.C., Granger, J.P. 2014. The heme 461 oxygenases: important regulators of pregnancy and preeclampsia. Am. J. Physiol. Regul. Integr. 462 Comp. Physiol. 307:R769-77. http://doi:10.1152/ajpregu.00132.2014. PMID:24898840. 463 464 Harris, C.B., Chowanadisai, W., Mishchuk, D.O., Satre, M.A., Slupsky, C.M., Rucker, R.B. 465 2013. Dietary pyrroloquinoline quinone (PQQ) alters indicators of inflammation and 466 mitochondrial-related metabolism in humanDraft subjects. J. Nutr. Biochem. 24:2076-84. 467 http://doi:10.1016/j.jnutbio.2013.07.008. PMID:24231099. 468 469 Ismailova, A., Kuter, D., Bohle, D.S., Butler, I.S. 2018. An Overview of the Potential 470 Therapeutic Applications of CO-Releasing Molecules. Bioinorg. Chem. Appl. Aug 471 12;2018:8547364. http://doi: 10.1155/2018/8547364. eCollection 2018. 472 473 Juárez, O., Nilges, M.J., Gillespie, P., Cotton, J., Blanca Barquera, B. 2008. Riboflavin is an 474 active redox in the Na+-pumping NADH:Quinone Oxidoreductase (Na+-NQR) from 475 Vibrio cholerae. J. Biol. Chem. 283: 33162–33167. http://doi: 10.1074/jbc.M806913200 476 477 Kim, H.H., Choi, S. 2018. Therapeutic aspects of carbon monoxide in cardiovascular disease. 478 Int. J. Mol. Sci. 19. pii: E2381. http://doi: 10.3390/ijms19082381. 479 480 Kinobe, R.T., Vlahakis, J.Z., Vreman, H.J., Stevenson, D.K., Brien, J.F., Szarek, W.A., Nakatsu, 481 K. 2006. Selectivity of imidazole-dioxolane compounds for in vitro inhibition of microsomal 482 haem oxygenase isoforms. Br. J. Pharmacol. 147:307-15. http://doi:10.1038/sj.bjp.0706555. 483 PMID:16331285. 484 485 Kumazawa T, Seno H, Urakami T, Matsumoto T, Suzuki O. Trace levels of pyrroloquinoline 486 quinone in human and rat samples detected by gas chromatography/mass spectrometry. Biochim. 487 Biophys. Acta. 1992;1156:62-6. http://doi:10.1016/0304-4165(92)90096-d. PMID:1335286. 488 489 Levytska, K., Kingdom, J., Baczyk, D., Drewlo, S. 2013. Heme oxygenase-1 in placental 490 development and pathology. Placenta. 34:291-8. http://doi:10.1016/j.placenta.2013.01.004. 491 PMID:23403148.

https://mc06.manuscriptcentral.com/cjpp-pubs Page 21 of 28 Canadian Journal of Physiology and Pharmacology

21

492 493 Lim, D., Morgan, R.J. Jr., Akman, S., Margolin, K., Carr, B.I., Leong, L., Odujinrin, O., 494 Doroshow, J.H. 2005. Phase I trial of menadiol diphosphate (vitamin K3) in advanced 495 malignancy. Invest. New Drugs. 23:235-9. http://doi:10.1007/s10637-005-6731-2. 496 PMID:15868379. 497 498 Maharshak, N., Ryu, H.S., Fan, T.-J., Onyiah, J.C., Schulz, S., Otterbein, S.L., Wong, R., 499 Hansen, J., Otterbein, L.E., Carroll, I., Plevy, S.E. 2015. Escherichia coli heme oxygenase 500 modulates host innate immune responses. Microbiol. Immunol. 59:452-465. 501 http://doi: 10.1111/1348-0421.12282. PMID: 26146866. 502 503 Maines, MD. 1997. The heme oxygenase system: a regulator of second messenger gases. Annu. 504 Rev. Pharmacol. Toxicol. 37:517-54. http://doi:10.1146/annurev.pharmtox.37.1.517. 505 PMID:9131263. 506 507 McNulty, H., Strain, J.J., Hughes, C.F., Ward, M. 2017. Riboflavin, MTHFR genotype and blood 508 pressure: A personalized approach to prevention and treatment of hypertension. Mol. Aspects. 509 Med. 53:2-9. http://doi:10.1016/j.mam.2016.10.002. PMID:27720779. 510 511 Misra, H.S., Rajpurohit, Y.S. Khairnar, N.P. 2012. Pyrroloquinoline-quinone and its versatile 512 roles in biological processes. J. Biosci. 37Draft:313–325. http://doi:10.1007/s12038-012-9195-5. 513 PMID:22581337. 514 515 Motterlini, R., Foresti, R. 2017. Biological signaling by carbon monoxide and carbon monoxide- 516 releasing molecules (CO-RMs). Am. J. Physiol. Cell. Physiol. 312: C302-C313. 517 http://doi:10.1152/ajpcell.00360.2016 PMID:31071151. 518 519 Neugebauer, J., Zanré, Y., Wacker, J. 2006. Riboflavin supplementation and preeclampsia. Int. J. 520 Gynecol. Obstet. 93:136-7. http://doi:10.1016/j.ijgo.2006.01.007. PMID:16545814. 521 522 Odozor, C.U., Peterson, N., Pudwell, J., Smith, G.N. 2018. Endogenous carbon monoxide 523 production by menadione. Placenta 71:6-12. http://doi:10.1016/j.placenta.2018.09.007. 524 PMID:30415746. 525 526 Phipps, E.A., Thadhani, R., Benzing, T., Karumanchi, S.A. 2019. Pre-eclampsia: pathogenesis, 527 novel diagnostics and therapies. Nat. Rev. Nephrol. 15: 275-289. http://doi:10.1038/s41581-019- 528 0119-6. PMID: 30792480. 529 530 Powers, H.J. 2003. Riboflavin (vitamin B-2) and health. Am. J. Clin. Nutr. 77:1352-1360. 531 http://doi:10.1093/ajcn/77.6.1352. PMID:12791609. 532 533 Ramma, W., Ahmed, A. 2014. Therapeutic potential of statins and the induction of heme 534 oxygenase-1 in preeclampsia. J. Reprod. Immunol. 101-102:153-160. 535 http://doi:10.1016/j.jri.2013.12.120. PMID:24503248. 536

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 22 of 28

22

537 Riddick, D.S., Ding, X., Wolf, C.R., Porter, T.D., Pandey, A.V., Zhang, Q.Y., Gu, J., Finn, R.D., 538 Ronseaux, S., McLaughlin, L.A., Henderson, C.J., Zou, L., Flück, C.E. 2013. NADPH- 539 cytochrome P450 oxidoreductase: roles in physiology, pharmacology, and toxicology. Drug 540 Metab. Dispos. 41:12-23. http://doi:10.1124/dmd.112.048991. PMID:23086197. 541 542 Suits, M. D., Pal, G. P., Nakatsu, K., Matte, A., Cygler, M., and Jia, Z. (2005) Proc. Natl. Acad. 543 Sci. U. S. A.102, 16955-16960 544 545 Thakur, K., Tomar, S.K., Singh, A.K., Manda, S., Arora, S. 2017. Riboflavin and health: A 546 review of recent human research. Crit. Rev. Food Sci. Nutr. 57:17:3650-3660. 547 http://doi:10.1080/10408398.2016.1145104. PMID:27029320. 548 549 Trakshel, G.M., Maines, M.D. 1988. Detection of two heme oxygenase isoforms in the human 550 testis. Biochem. Biophys. Res. Commun. 154:285-91. http://doi:10.1016/0006-291x(88)90682-1. 551 PMID:3395332. 552 553 Uniprot.org; https://www.uniprot.org/uniref/UniRef100_A0A0K3Z2M4. Accessed 2019-10-15. 554 555 Venditti C.C., Casselman, R., Young, I., Karumanchi, S.A., Smith, G.N. 2014. Carbon monoxide 556 prevents hypertension and proteinuria in an adenovirus sFlt-1 preeclampsia-like mouse model. 557 PLOS One 9:1–7. http://doi:10.1371/journal.pone.0106502.Draft PMID:25202912. 558 559 Venditti, C.C., Casselman, R., Murphy, M.S.Q., Adamson, S.L., Sled, J.G., Smith, G.N. 2013. 560 Chronic carbon monoxide inhalation during pregnancy augments uterine artery blood flow and 561 uteroplacental vascular growth in mice. Am. J. Physiol. Regul. Integr. Comp. Physiol. 305:R939- 562 R948. http://doi:10.1152/ajpregu.00204.2013. PMID:23986360. 563 564 Venditti, C.C., Smith, G.N. 2014. Involvement of the heme oxygenase system in the 565 development of preeclampsia and as a possible therapeutic target. Women’s Health (Lond). 566 10:623-43. http://doi:10.2217/whe.14.54. PMID:25482489. 567 568 Vreman, H.J., Stevenson, D.K. 1988. Heme oxygenase activity as measured by carbon monoxide 569 production. Anal. Biochem. 168:31–38. 570 571 Vukomanovic, D., McLaughlin, B.E., Rahman, M.N., Szarek, W.A., Brien, J.F., Jia, Z., Nakatsu, 572 K. 2011. Selective activation of heme oxygenase-2 by menadione. Can. J Physiol. Pharmacol. 573 89:861–864. http://doi:10.1139/Y11-091. PMID:22039914. 574 575 Vukomanovic, D., McLaughlin, B., Rahman, M.N., Vlahakis, J.Z., Roman, G., Dercho, R.A., 576 Kinobe, R.T., Hum, M., Brien, J.F., Jia, Z., Szarek, W.A., Nakatsu, K. 2010. Recombinant 577 truncated and microsomal heme oxygenase-1 and -2: differential sensitivity to inhibitors. Can. J. 578 Physiol. Pharmacol. 88:480-6. http://doi:10.1139/y10-004. PMID:20555417. 579 580 Vukomanovic, D., Rahaman, M.N., Bilokin, Y., Golub, A.G., Brien, J.F., Szarek, W.A., Jia, Z., 581 Nakatsu, K. 2014. In Vitro Activation of heme oxygenase-2 by menadione and its analogs. Med. 582 Gas. Res. 4:1-11. http://doi:10.1186/2045-9912-4-4. PMID:24533775.

https://mc06.manuscriptcentral.com/cjpp-pubs Page 23 of 28 Canadian Journal of Physiology and Pharmacology

23

583 584 Vukomanovic, D., Rahman, M., Jia, Z., Nakatsu, K. 2017. Drug-enhanced carbon monoxide 585 production from heme by cytochrome P450 reductase. Med. Gas. Res. 7: 37-4. 586 http://doi:10.4103/2045-9912.202908. PMID: 28480030. 587 588 Wacker, J., Frühauf, J., Schulz, M., Chiwora, F.M., Volz, J., Becker, K. 200. Riboflavin 589 deficiency and preeclampsia. Obstet. Gynecol. 96:38-44. PMID:1086283. 590 591 Zorn, J.A., Wells, J.A. 2010. Turning enzymes ON with small molecules. Nat. Chem. Biol. 592 6:179-188. http://doi:10.1038/nchembio.318. PMID:20154666. 593 594

595 FIGURE LEGENDS

596

597 Figure 1. Enhanced CO production by rat spleen microsomes in the presence of riboflavin 598 (RB) and pyrolloquinolone-quinone (PQQ).Draft Heme degradation in microsomal fractions (1 mg 599 of protein) prepared from four different male rat spleens (triplicates for each) was quantified by

600 measurement of CO using a headspace gas chromatography method, after 15 minutes of

601 incubation with RB or PQQ and 1.0 mM NADPH in 100 mM phosphate buffer (pH = 7.4) at

602 37°C. Data are presented as mean ± standard deviation, picomoles of CO (pmol CO). The

603 asterisk denotes a statistically significant difference of at least p< 0.05 from the respective RB or

604 PQQ value at 10 nM.

605

606 Figure 2. Enhanced CO production by rat brain microsomes in the presence of riboflavin

607 (RB) and pyrolloquinoline-quinone (PQQ). Heme degradation in microsomal fractions (1 mg

608 of protein) from four different male rat brains (triplicates for each) was quantified by

609 measurement of CO using a headspace gas chromatography method, after 15 minutes of

610 incubation with RB or PQQ and 1.0 mM NADPH in 100 mM phosphate buffer (pH = 7.4) at

611 37°C. Data are presented as mean ± standard deviation, picomoles of CO (pmol CO). The

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 24 of 28

24

612 asterisk denotes a statistically significant difference of at least p< 0.05 from the respective RB or

613 PQQ value at 10 nM.

614 Figure 3. Enhanced CO production by human placenta microsomes in the presence of

615 riboflavin (RB) and pyrolloquinolone-quinone (PQQ). Heme degradation in microsomal

616 fractions (1 mg of protein) from four different placentas (triplicates for each) was quantified by

617 measurement of CO using a headspace gas chromatography method, after 15 minutes of

618 incubation with RB or PQQ and 1.0 mM NADPH in 100 mM phosphate buffer (pH = 7.4) at

619 37°C. Data are presented as mean ± standard deviation, picomoles of CO (pmol CO). The

620 asterisk denotes a statistically significant difference of at least p< 0.05 from the respective RB or

621 PQQ value at 10 nM.

622 Draft

623 Figure 4. Pyrolloquinoline-quinone (PQQ) produces significantly more CO than riboflavin

624 (RB) in the presence of recombinant human CPR (rh-CPR). Panel A); the heme degradation

625 in the presence of 10 μM rh-CPR (four replicates from the same preparation) was quantified by

626 measurement of CO using a headspace gas chromatography method, after 15 minutes of

627 incubation with RB or PQQ and 1.0 mM NADPH in 100 mM phosphate buffer (pH = 7.4) at

628 37°C. Data are presented as mean ± standard deviation, picomoles of CO (pmol CO). The

629 asterisk denotes a statistically significant difference of at least p< 0.05 from the respective RB or

630 PQQ value at 10 nM. Panel B); The SDS PAGE (10%) of the rh-CPR preparation used to

631 generate CO in panel A) was stained with Coomasie Brilliant Blue. Lane 1 contains a molecular

632 weight ladder and Lane 2 the rh-CPR-containing preparation. The arrowhead denotes the

633 location of rh-CPR.

634

https://mc06.manuscriptcentral.com/cjpp-pubs Page 25 of 28 Canadian Journal of Physiology and Pharmacology

Draft

Figure 1. Enhanced CO production by rat spleen microsomes in the presence of riboflavin (RB) and pyrolloquinolone-quinone (PQQ). Heme degradation in microsomal fractions (1 mg of protein) prepared from four different male rat spleens (triplicates for each) was quantified by measurement of CO using a headspace gas chromatography method, after 15 minutes of incubation with RB or PQQ and 1.0 mM NADPH in 100 mM phosphate buffer (pH = 7.4) at 37°C. Data are presented as mean ± standard deviation, picomoles of CO (pmol CO). The asterisk denotes a statistically significant difference of at least p< 0.05 from the respective RB or PQQ value at 10 nM.

254x190mm (72 x 72 DPI)

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 26 of 28

Draft

Figure 2. Enhanced CO production by rat brain microsomes in the presence of riboflavin (RB) and pyrolloquinoline-quinone (PQQ). Heme degradation in microsomal fractions (1 mg of protein) from four different male rat brains (triplicates for each) was quantified by measurement of CO using a headspace gas chromatography method, after 15 minutes of incubation with RB or PQQ and 1.0 mM NADPH in 100 mM phosphate buffer (pH = 7.4) at 37°C. Data are presented as mean ± standard deviation, picomoles of CO (pmol CO). The asterisk denotes a statistically significant difference of at least p< 0.05 from the respective RB or PQQ value at 10 nM.

254x190mm (72 x 72 DPI)

https://mc06.manuscriptcentral.com/cjpp-pubs Page 27 of 28 Canadian Journal of Physiology and Pharmacology

Draft

Figure 3. Enhanced CO production by human placenta microsomes in the presence of riboflavin (RB) and pyrolloquinolone-quinone (PQQ). Heme degradation in microsomal fractions (1 mg of protein) from four different placentas (triplicates for each) was quantified by measurement of CO using a headspace gas chromatography method, after 15 minutes of incubation with RB or PQQ and 1.0 mM NADPH in 100 mM phosphate buffer (pH = 7.4) at 37°C. Data are presented as mean ± standard deviation, picomoles of CO (pmol CO). The asterisk denotes a statistically significant difference of at least p< 0.05 from the respective RB or PQQ value at 10 nM.

254x190mm (72 x 72 DPI)

https://mc06.manuscriptcentral.com/cjpp-pubs Canadian Journal of Physiology and Pharmacology Page 28 of 28

Draft

Figure 4. Pyrolloquinoline-quinone (PQQ) produces significantly more CO than riboflavin (RB) in the presence of recombinant human CPR (rh-CPR). Panel A); the heme degradation in the presence of 10 μM rh-CPR (four replicates from the same preparation) was quantified by measurement of CO using a headspace gas chromatography method, after 15 minutes of incubation with RB or PQQ and 1.0 mM NADPH in 100 mM phosphate buffer (pH = 7.4) at 37°C. Data are presented as mean ± standard deviation, picomoles of CO (pmol CO). The asterisk denotes a statistically significant difference of at least p< 0.05 from the respective RB or PQQ value at 10 nM. Panel B); The SDS PAGE (10%) of the rh-CPR preparation used to generate CO in panel A) was stained with Coomasie Brilliant Blue. Lane 1 contains a molecular weight ladder and Lane 2 the rh-CPR-containing preparation. The arrowhead denotes the location of rh-CPR.

254x190mm (72 x 72 DPI)

https://mc06.manuscriptcentral.com/cjpp-pubs