Fructose As an Endogenous Toxin
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Clinical Applications and Implications of Common and Founder Mutations in Indian Subpopulations
REVIEW OFFICIAL JOURNAL Clinical Applications and Implications of Common and Founder Mutations in Indian Subpopulations www.hgvs.org Arunkanth Ankala,1∗ † Parag M. Tamhankar,2 † C. Alexander Valencia,3,4 Krishna K. Rayam,5 Manisha M. Kumar,5 and Madhuri R. Hegde1 1Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia; 2ICMR Genetic Research Center, National Institute for Research in Reproductive Health, Mumbai, Maharashtra, India; 3Division of Human Genetics, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio; 4Department of Pediatrics, University of Cincinnati Medical School, Cincinnati, Ohio; 5Department of Biosciences, CMR Institute of Management Studies, Bangalore, Karnataka, India Communicated by Arupa Ganguly Received 24 October 2013; accepted revised manuscript 16 September 2014. Published online 27 November 2014 in Wiley Online Library (www.wiley.com/humanmutation). DOI: 10.1002/humu.22704 that include a lack of widespread awareness about genetic disorders in the general population and the scarcity of specialized medical ABSTRACT: South Asian Indians represent a sixth of the world’s population and are a racially, geographically, and professionals and affordable genetic tests. Seeking a molecular di- genetically diverse people. Their unique anthropological agnosis and understanding the risk estimates are critical to making structure, prevailing caste system, and ancient religious sound reproductive choices, especially in families with an affected practices have all impacted the genetic composition of most individual. Adding to this adversity is the absence of a properly func- of the current-day Indian population. With the evolving tioning social health care system and insufficient encouragement socio-religious and economic activities of the subsects and of individual health insurance by the government. -
Glucose Oxidase
Catalog Number: 100289, 100330, 195196 Glucose Oxidase Molecular Weight: ~160,0001 CAS #: 9001-34-0 Physical Description: Yellow lyophilized powder Source: Aspergillus niger Isoelectric Point: 4.2 Michaelis Constants: phosphate buffer, pH 5.6; 25°C, air: Glucose: 3.3 x 10-2 mol/l(2) Glucose: 1.1 x 10-1 mol/l(3) 2-Deoxyglucose: 2.5 x 10-2 mol/l O2: 2.0 x 10-4 mol/l Structure: The enzyme contains 2 moles FAD/mole GOD. Inhibitors: Ag+, Hg2+, Cu2+ (4), 4-chloromercuribenzoate, D-arabinose (50%). FAD binding is inhibited by several nucleotides.5 pH Optimum: 6.5 pH Stability: 8.0 pH Range: 4-7 Thermal Stability: Below 40°C Description: Glucose oxidase is an FAD-containing glycoprotein. The enzyme is specific for b-D-glucose. O2 can be replaced by hydrogen acceptors such as 2.6-dichlorophenol indophenol. Relative Rates: D-glucose, 100; D-mannose, 20; 2-Deoxy-D-glucose, 20; negligible on other hexoses. Solubility: Dissolves readily at 5 mg/ml in 0.1 M potassium phosphate pH 7.0, giving a clear, yellow solution, also soluble in water Assay Procedure 1: Unit Definition: One unit of glucose oxidase is the activity which causes the liberation of 1 micromole of H2O2 per minute at 25°C and pH 7.0 under the specified conditions. Reaction: Reagents: 1. 0.1 M Phosphate Buffer, pH 6.8: dissolve 6.8 g of potassium phosphate, monobasic, anhydrous and 7.1 g of sodium phosphate, dibasic, anhydrous in about 800 ml of deionized water. Adjust the pH to 6.8 ± 0.05 @ 25°C with 1 N HCl or 1N NaOH if necessary. -
Oxalate Loading Test: a Screening Test for Steatorrhoea
Gut: first published as 10.1136/gut.20.12.1089 on 1 December 1979. Downloaded from Gut, 1979, 20, 1089-1094 Oxalate loading test: a screening test for steatorrhoea D. S. RAMPTON', G. P. KASIDAS, G. ALAN ROSE, AND MARTIN SARNER2 From University College Hospital, London, St. Peter's Hospitals and Institute of Urology, London SUMMARY To investigate the possibility of measuring urinary oxalate output instead of faecal fat excretion as an outpatient screening test for steatorrhoea, we determined 24 hour urinary oxalate and five day faecal fat excretion before and during an oral load of sodium oxalate 600 mg daily (oxalate 4-44 mmol), in 32 patients with suspected malabsorption on a diet containing oxalate 30 mg (0-33 mmol), fat 50 g (180 mmol), and calcium 1 g (25 mmol). Nineteen patients proved to have steatorrhoea (mean faecal fat 62 mmol/24 h, range 19-186 mmol) of varying aetiologies. On the diet alone, urinary oxalate was raised in only nine of these patients (mean 0 25 mmol/24 h, range 0-08-059 mmol) (normal <0 20). By contrast, when the diet was supplemented with oral sodium oxalate, all 19 patients with steatorrhoea had hyperoxaluria (mean 0-91 mmol/24 h, range 046- 1P44 mmol) (normal <0-44). There was a significant positive linear relationship between urinary oxalate and faecal fat when the 32 patients were on the high oxalate intake (r=0*73, P <0.001), but not when they were on the low oxalate intake. Mean percentage absorption of orally administered oxalate was 58+09% (±1 SD) in normal subjects and 14-7+6-0% (P <0.002) in patients with steatorrhoea. -
A New Insight Into Role of Phosphoketolase Pathway in Synechocystis Sp
www.nature.com/scientificreports OPEN A new insight into role of phosphoketolase pathway in Synechocystis sp. PCC 6803 Anushree Bachhar & Jiri Jablonsky* Phosphoketolase (PKET) pathway is predominant in cyanobacteria (around 98%) but current opinion is that it is virtually inactive under autotrophic ambient CO2 condition (AC-auto). This creates an evolutionary paradox due to the existence of PKET pathway in obligatory photoautotrophs. We aim to answer the paradox with the aid of bioinformatic analysis along with metabolic, transcriptomic, fuxomic and mutant data integrated into a multi-level kinetic model. We discussed the problems linked to neglected isozyme, pket2 (sll0529) and inconsistencies towards the explanation of residual fux via PKET pathway in the case of silenced pket1 (slr0453) in Synechocystis sp. PCC 6803. Our in silico analysis showed: (1) 17% fux reduction via RuBisCO for Δpket1 under AC-auto, (2) 11.2–14.3% growth decrease for Δpket2 in turbulent AC-auto, and (3) fux via PKET pathway reaching up to 252% of the fux via phosphoglycerate mutase under AC-auto. All results imply that PKET pathway plays a crucial role under AC-auto by mitigating the decarboxylation occurring in OPP pathway and conversion of pyruvate to acetyl CoA linked to EMP glycolysis under the carbon scarce environment. Finally, our model predicted that PKETs have low afnity to S7P as a substrate. Metabolic engineering of cyanobacteria provides many options for producing valuable compounds, e.g., acetone from Synechococcus elongatus PCC 79421 and butanol from Synechocystis sp. strain PCC 68032. However, certain metabolites or overproduction of intermediates can be lethal. Tere is also a possibility that required mutation(s) might be unstable or the target bacterium may even be able to maintain the fux distribution for optimal growth balance due to redundancies in the metabolic network, such as alternative pathways. -
Etude Des Sources De Carbone Et D'énergie Pour La Synthèse Des Lipides De Stockage Chez La Microalgue Verte Modèle Chlamydo
Aix Marseille Université L'Ecole Doctorale 62 « Sciences de la Vie et de la Santé » Etude des sources de carbone et d’énergie pour la synthèse des lipides de stockage chez la microalgue verte modèle Chlamydomonas reinhardtii Yuanxue LIANG Soutenue publiquement le 17 janvier 2019 pour obtenir le grade de « Docteur en biologie » Jury Professor Claire REMACLE, Université de Liège (Rapporteuse) Dr. David DAUVILLEE, CNRS Lille (Rapporteur) Professor Stefano CAFFARRI, Aix Marseille Université (Examinateur) Dr. Gilles PELTIER, CEA Cadarache (Invité) Dr. Yonghua LI-BEISSON, CEA Cadarache (Directeur de thèse) 1 ACKNOWLEDGEMENTS First and foremost, I would like to express my sincere gratitude to my advisor Dr. Yonghua Li-Beisson for the continuous support during my PhD study and also gave me much help in daily life, for her patience, motivation and immense knowledge. I could not have imagined having a better mentor. I’m also thankful for the opportunity she gave me to conduct my PhD research in an excellent laboratory and in the HelioBiotec platform. I would also like to thank another three important scientists: Dr. Gilles Peltier (co- supervisor), Dr. Fred Beisson and Dr. Pierre Richaud who helped me in various aspects of the project. I’m not only thankful for their insightful comments, suggestion, help and encouragement, but also for the hard question which incented me to widen my research from various perspectives. I would also like to thank collaboration from Fantao, Emmannuelle, Yariv, Saleh, and Alisdair. Fantao taught me how to cultivate and work with Chlamydomonas. Emmannuelle performed bioinformatic analyses. Yariv, Saleh and Alisdair from Potsdam for amino acid analysis. -
Blueprint Genetics Primary Hyperoxaluria Panel
Primary Hyperoxaluria Panel Test code: KI0801 Is a 3 gene panel that includes assessment of non-coding variants. Is ideal for patients with a clinical suspicion of hyperoxaluria. About Primary Hyperoxaluria The primary hyperoxalurias are rare disorders of glyoxylate metabolism, which result in markedly increased endogenous oxalate synthesis by the liver. They are characterized by an excess of oxalate resulting in manifestations ranging from occasional renal stones, recurrent nephrolithiasis and nephrocalcinosis to end-stage renal disease (ESRD) and systemic oxalosis. Presenting ranges from the neonatal period to adulthood. Among disorders causing hyperoxaluria, the primary hyperoxalurias are the most severe, ultimately leading to ESRD and if untreated, death in most patients. Type I primary hyperoxaluria (PH1), is caused by deficient or absent activity of liver-specific peroxisomal alanine glyoxylate aminotransferase (AGT). In some patients with PH1 type disease, the enzyme is present but mistargeted to mitochondria where it is metabolically inactive. The severe infantile form is characterized by a failure to thrive, nephrocalcinosis with or without nephrolithiasis and early ESRD. Onset in childhood and adolescence is often characterized by recurrent urolithiasis (with or without nephrocalcinosis) and progressive renal failure. The late onset form is characterized by occasional renal stones with onset in adulthood, but acute renal failure caused by bilateral obstruction of the kidneys by oxalate stones may occur. Other manifestations include urinary tract infections, dysuria and hematuria. The ongoing systemic oxalosis also may lead to other clinical manifestations such as cardiac conduction defects, vascular calcification with distal gangrene, disturbed vision, specific brown colored retinal deposits, skin nodules, joint involvement and bone disease leading to fractures in long-term dialysis-dependent patients. -
Redalyc.The Nutritional Limitations of Plant-Based Beverages in Infancy
Nutrición Hospitalaria ISSN: 0212-1611 [email protected] Sociedad Española de Nutrición Parenteral y Enteral España Vitoria, Isidro The nutritional limitations of plant-based beverages in infancy and childhood Nutrición Hospitalaria, vol. 34, núm. 5, 2017, pp. 1205-1214 Sociedad Española de Nutrición Parenteral y Enteral Madrid, España Available in: http://www.redalyc.org/articulo.oa?id=309253341026 How to cite Complete issue Scientific Information System More information about this article Network of Scientific Journals from Latin America, the Caribbean, Spain and Portugal Journal's homepage in redalyc.org Non-profit academic project, developed under the open access initiative Nutr Hosp. 2017; 34(5):1205-1214 ISSN 0212-1611 - CODEN NUHOEQ S.V.R. 318 Nutrición Hospitalaria Revisión The nutritional limitations of plant-based beverages in infancy and childhood Limitaciones nutricionales de las bebidas vegetales en la lactancia y la infancia Isidro Vitoria Unit of Nutrition and Metabolopathies. Hospital Universitario y Politécnico La Fe. Valencia, Spain Abstract Breastfeeding, infant formula and cow’s milk are basic foods in infant nutrition. However, they are being increasingly replaced either totally or partially by plant-based beverages. The composition of 164 plant-based beverages available in Spain was reviewed based on the nutritional labeling of the package and the man- ufacturers’ webpages. This was compared to the composition of cow’s milk and infant formula. In addition, the nutritional disease associated with consumption of plant-based beverages in infants and children was reviewed by means of a literature search in Medline and Embase since 1990 based on the key words “plant-based beverages” or “rice beverages” or “almond beverages” or “soy beverages” and “infant” or “child”. -
Peroxisomal Alanine:Glyoxylate Aminotransferase Deficiency in Primary Hyperoxaluria Type I
Volume 201, number 1 FEBS 3672 May 1986 Peroxisomal alanine:glyoxylate aminotransferase deficiency in primary hyperoxaluria type I C.J. Danpure and P.R Jennings Divisum of Inherited Metabolic Diseases, Clinical Research Centre, Watford Road, Harrow HA1 3UJ, England Received 1 April 1986 Activities of alanine:glyoxylate aminotransferase in the livers of two patients with primary hyperoxaluria type I were substantially lower than those found in five control human livers. Detailed subcellular fractiona- tion of one of the hyperoxaluric livers, compared with a control liver, showed that there was a complete absence of peroxisomal alanine:glyoxylate aminotransferase. This enzyme deficiency explains most of the biochemical characteristics of the disease and means that primary hyperoxaluria type I should be added to the rather select list of peroxisomal disorders. Hyperoxaluria Alanine:glyoxylate aminotransferase Glutamate:glyoxylate aminotransferase Peroxisomal disorder Glyoxylate metabolism (Human) Liver pathology 1. INTRODUCTION transamination in primary hyperoxaluria type I. Our results suggest that the basic biochemical Primary hyperoxaluria type I is a rare inborn er- defect in the disease is the absence of peroxisomal ror of metabolism caused by an accumulation of alanine : glyoxylate aminotransferase. glyoxylate, which leads to increased synthesis and excretion of oxalate and glycolate. Clinically the disease is characterized by recurrent calcium ox- 2. EXPERIMENTAL alate kidney stones, resulting in progressive renal insufficiency and death usually before the age of 2.1. Livers 20 [l]. Numerous in vivo studies in the 1960s sug- The subcellular fractionation experiments were gested that there might be an abnormality in the carried out on the liver of a patient with transamination of glyoxylate to glycine [2-41 in pyridoxine-resistant primary hyperoxaluria type I the type I disease, but the observations made in and a normal human liver. -
Optimization of Immobilized Aldose Reductase Isolated from Bovine Liver Sığır Karaciğerinden İzole Edilen İmmobilize Aldoz Redüktazın Optimizasyonu
Turk J Pharm Sci 2019;16(2):206-210 DOI: 10.4274/tjps.galenos.2018.81894 ORIGINAL ARTICLE Optimization of Immobilized Aldose Reductase Isolated from Bovine Liver Sığır Karaciğerinden İzole Edilen İmmobilize Aldoz Redüktazın Optimizasyonu Marya Vakıl NASLIYAN, Sidar BEREKETOĞLU, Özlem YILDIRIM* Ankara University, Faculty of Science, Department of Biology, Ankara, Turkey ABSTRACT Objectives: Isolation of enzymes and experiments on them require great effort and cost and are time-consuming. Therefore, it is important to extend the usability of the enzymes by immobilizing them. In this study our purpose was to immobilize the enzyme aldose reductase (AR) and to optimize the experimental conditions of the immobilized AR and compare them to those of free AR. Materials and Methods: AR was isolated from bovine liver and the enzyme immobilized in photographic gelatin by cross-linking with glutaraldehyde. Then the optimum conditions for free and immobilized AR in terms of pH, temperature, and storage were characterized by determining the enzyme activity. Results: Following immobilization, the optimum pH and temperature levels for free AR, which were pH 7.0 and 60°C, slightly altered to pH 7.5 and 50°C. The enzyme activity of the immobilized AR was maintained at about 65% after reusing 15 times. Moreover, immobilized AR maintained 95% of its original activity after 20 days of storage at 4°C, while the retained activity of the free AR was 85% of the original. Conclusion: Our experiments indicated that the conditions that affect enzyme activity might alter following immobilization. Once the optimum experimental conditions are fixed, the immobilized AR can be stored and reused with efficiency higher than that of free AR. -
A High-Throughput Screening System Based on Droplet Microfluidics For
molecules Article A High-Throughput Screening System Based on Droplet Microfluidics for Glucose Oxidase Gene Libraries Radivoje Prodanovi´c 1,2,* , W. Lloyd Ung 2, Karla Ili´c Đurđi´c 1 , Rainer Fischer 3, David A. Weitz 2 and Raluca Ostafe 4 1 Faculty of Chemistry, University of Belgrade, Studentski trg 12, 11000 Belgrade, Serbia; [email protected] 2 Department of Physics, School of Engineering and Applied Sciences, Harvard University, Cambridge, MA 02138, USA; [email protected] (W.L.U.); [email protected] (D.A.W.) 3 Departments of Biological Sciences and Chemistry, Purdue University, 207 S. Martin Jischke Dr., West Lafayette, IN 47907, USA; fi[email protected] 4 Purdue Institute of Inflammation, Immunology and Infectious Disease, Molecular Evolution, Protein Engineering and Production, Purdue University, 207 S. Martin Jischke Dr., West Lafayette, IN 47907, USA; [email protected] * Correspondence: [email protected]; Tel.: +38-111-333-6660 Academic Editors: Goran T. Vladisavljevi´cand Guido Bolognesi Received: 20 April 2020; Accepted: 15 May 2020; Published: 22 May 2020 Abstract: Glucose oxidase (GOx) is an important industrial enzyme that can be optimized for specific applications by mutagenesis and activity-based screening. To increase the efficiency of this approach, we have developed a new ultrahigh-throughput screening platform based on a microfluidic lab-on-chip device that allows the sorting of GOx mutants from a saturation mutagenesis library expressed on the surface of yeast cells. GOx activity was measured by monitoring the fluorescence of water microdroplets dispersed in perfluorinated oil. The signal was generated via a series of coupled enzyme reactions leading to the formation of fluorescein. -
The Genome of an Industrial Workhorse
NEWS AND VIEWS The genome of an industrial workhorse Dan Cullen Sequencing of the filamentous fungus Aspergillus niger offers new opportunities for the production of specialty chemicals and enzymes. Few microbes compare with the filamentous fungus Aspergillus niger in its ability to pro Environment CAT (2) H O H O + O duce prodigious amounts of useful chemicals 2 2 2 2 and enzymes. This fungus is the principal GOX (3) GLN (1) source of citric acid for food, beverages and D-glucono Glucose Gluconate Oxalate pharmaceuticals1 and of several important 1,5-lactone Citrate http://www.nature.com/naturebiotechnology http://www.nature.com/naturebiotechnology commercial enzymes, including glucoamy lase, which is widely used for the conversion of starch to food syrups and to fermentative Oxalate + acetate PEP feedstocks for ethanol production. Although OAH most of these fermentation processes are well cMDH (3) established, the underlying genetics are still cPYC (1) cACO (2) cIDH (1) poorly understood. In this issue, Pel et al.2 Pyruvate OAA MAL Citrate Isocitrate 2-ketoglutarate report the genome sequence of A. niger strain Cytosol CBS 513.88. The availability of this sequence OAT (1) CMC (2) mPYC (1) should provide invaluable aid toward improv Pyruvate OAA MAL Citrate Isocitrate 2-ketoglutarate ing the production of chemicals and enzymes PDH Nature Publishing Group Group Nature Publishing 7 in this organism. mMDH (1) mACO (2) mIDH (3) Pel et al. sequenced tiled bacterial artificial CS (3) 200 Acetyl-CoA TCA cycle © chromosomes representing the entire A. niger genome to produce a high-quality assembly of Mitochondrion 19 supercontigs with a combined length of 33.9 Mb. -
Antenatal Diagnosis of Inborn Errors Ofmetabolism
816 ArchivesofDiseaseinChildhood 1991;66: 816-822 CURRENT PRACTICE Arch Dis Child: first published as 10.1136/adc.66.7_Spec_No.816 on 1 July 1991. Downloaded from Antenatal diagnosis of inborn errors of metabolism M A Cleary, J E Wraith The introduction of experimental treatment for Sample requirement and techniques used in lysosomal storage disorders and the increasing prenatal diagnosis understanding of the molecular defects behind By far the majority of antenatal diagnoses are many inborn errors have overshadowed the fact performed on samples obtained by either that for many affected families the best that can amniocentesis or chorion villus biopsy. For be offered is a rapid, accurate prenatal diag- some disorders, however, the defect is not nostic service. Many conditions remain at best detectable in this material and more invasive only partially treatable and as a consequence the methods have been applied to obtain a diagnos- majority of parents seek antenatal diagnosis in tic sample. subsequent pregnancies, particularly for those disorders resulting in a poor prognosis in terms of either life expectancy or normal neurological FETAL LIVER BIOPSY development. Fetal liver biopsy has been performed to The majority of inborn errors result from a diagnose ornithine carbamoyl transferase defi- specific enzyme deficiency, but in some the ciency and primary hyperoxaluria type 1. primary defect is in a transport system or Glucose-6-phosphatase deficiency (glycogen enzyme cofactor. In some conditions the storage disease type I) could also be detected by biochemical defect is limited to specific tissues this method. The technique, however, is inva- only and this serves to restrict the material avail- sive and can be performed by only a few highly able for antenatal diagnosis for these disorders.