Aspartate Aminotransferase (AST, GOT), Human Liver

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05/18

For research use only

Aspartate Aminotransferase (AST, GOT), Human Liver

CATALOG NO:

P1299-100
1 units

RELATED PRODUCTS:

ALTERNATE NAMES:

Aspartate Transaminase, Glutamate Oxaloacetate, AST, GOT, sGOT, AspAT, ASAT, serum glutamic oxaloacetic transaminase, AAT



Aspartate Aminotransferase (AST) (Mouse) ELISA Kit (Cat. No. E4320) Aspartate Aminotransferase (AST) (Human) ELISA Kit (Cat. No. E4319) Aspartate Aminotransferase (AST) (Rat) ELISA Kit (Cat. No. E4321) Aspartate Aminotransferase (AST or SGOT) Activity Colorimetric Assay Kit (Cat.

No. K753)
SOURCE:

Human Liver

PURITY:

Purified



Anti-GOT2 Antibody (cat. No. A1273)
MOL. WEIGHT:

FORM:

~92 kDa

Anti-GOT1 Antibody (Cat. No. A1272) GOT2, human recombinant (Cat. No. 7809)

Lyophilized

GOT1, human recombinant (Cat. No. 7808)
STORAGE CONDITIONS:

BIOLOGICAL ACTIVITY: UNIT DEFINITATION:

Store at -20°C. Avoid repeated freezing and thawing cycles. ≥ 1 U/mg (Dimension® Clinical Chemistry System) One unit will catalyze the transamination of one micromole of L- aspartate to alpha-ketoglutarate forming L-glutamate and oxaloacetate per minute at 37°C and pH 7.8.Measured at 340 nm as one equimolar amount of NAD produced by a coupled reaction.

RECONSTITUTION: DESCRIPTION:

> 1 mg/mL in tris buffered saline, 1% BSA, pH 8.0. AST/SGOT is found in many tissues throughout the body, including the liver, heart, muscles, kidney, and brain. If any of these organs or tissues is affected by disease or injury, AST is released into the bloodstream. This means that AST isn't as specific an indicator of liver damage as ALT (also known as alanine aminotransferase, another type of enzyme found almost entirely in the liver). The ratio of AST and ALT levels are commonly used as biomarkers for liver health.

FOR RESEARCH USE ONLY! Not to be used on humans.

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Supplementary Materials

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Supporting Information

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Supplementary Table 2

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Anti-GOT2 Antibody (ARG41582)

Product datasheet [email protected] ARG41582 Package: 100 μl anti-GOT2 antibody Store at: -20°C Summary Product Description Rabbit Polyclonal antibody recognizes GOT2 Tested Reactivity Hu, Ms, Rat Tested Application IHC-P, WB Host Rabbit Clonality Polyclonal Isotype IgG Target Name GOT2 Antigen Species Human Immunogen Recombinant fusion protein corresponding to aa. 30-200 of Human GOT2 (NP_002071.2). Conjugation Un-conjugated Alternate Names Kynurenine aminotransferase 4; mitAAT; Plasma membrane-associated fatty acid-binding protein; Kynurenine--oxoglutarate transaminase 4; Glutamate oxaloacetate transaminase 2; KAT4; Transaminase A; KATIV; FABP-1; Aspartate aminotransferase, mitochondrial; FABPpm; Fatty acid- binding protein; Kynurenine--oxoglutarate transaminase IV; mAspAT; Kynurenine aminotransferase IV; EC 2.6.1.1; EC 2.6.1.7 Application Instructions Application table Application Dilution IHC-P 1:50 - 1:200 WB 1:500 - 1:2000 Application Note * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. Positive Control BT-474 Calculated Mw 48 kDa Observed Size ~ 43 kDa Properties Form Liquid Purification Affinity purified. Buffer PBS (pH 7.3), 0.02% Sodium azide and 50% Glycerol. Preservative 0.02% Sodium azide Stabilizer 50% Glycerol www.arigobio.com 1/2 Storage instruction For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. Note For laboratory research only, not for drug, diagnostic or other use.
Liver (Version 1).Xlsb

Supplemental Table 6: Sulfhydrated Protein Pathway Enrichment in Liver KEGG Pathway (DR) p‐val (adj) ‐LOG10(p‐val adj) Involved Genes/Proteins Metabolic pathways 0.0345 1.46 NDUFS6,HSD17B10,ACADL,NDUFA8,HMGCS2,UROD,ACSM1,PKLR,CES1C,PTGES3 KEGG Pathway (Unchanged) p‐val (adj) ‐LOG10(p‐val adj) Involved Genes/Proteins Pentose phosphate pathway 0.00639 2.19 ALDOC,TKT,RGN,TALDO1,ALDOB,RBKS,PGM1,GPI,FBP1 Bacterial invasion of epithelial cells 0.00658 2.18 CRKL,ARPC2,CDC42,RHOA,ARPC5,CRK,VCL,CLTA,ARPC3,ARPC1A,ARPC1B,CTTN,ACTG1,ARPC4 Peroxisome 0.000000196 6.71 SOD2,ACAA1B,PIPOX,ACOX1,HACL1,EPHX2,AMACR,SOD1,XDH,HSD17B4,PRDX5,IDH1,AGXT,PHYH,CAT,HAO1,SCP2,HMGCL,PRDX1,NUDT7,ACAA1A,ECH1 Glycine, serine and threonine metabolism 0.00000344 5.46 GNMT,SARDH,PGAM1,CHDH,PIPOX,SHMT1,DLD,CBS,GLDC,AGXT,CTH,GRHPR,DMGDH,ALDH7A1 Glutathione metabolism 0.00000136 5.87 GSTM3,SRM,MGST1,GPX3,OPLAH,GSTO1,GSTA3,IDH1,GSS,TXNDC12,GSR,GCLC,LAP3,GSTM2,GSTM1,GPX1,GSTA1,GPX4 Lysine degradation 0.00156 2.81 GCDH,DLST,PIPOX,OGDH,ACAT2,ECHS1,HADHA,ALDH9A1,HADH,ALDH2,ACAT1,ALDH7A1,TMLHE Protein processing in endoplasmic reticulum 0.00447 2.35 PRKCSH,RAD23A,CALR,DNAJB11,UFD1,HSPA8,CANX,PDIA6,EIF2S1,HSP90AB1,P4HB,PDIA4,HSPA5,PDIA3,RRBP1,VCP,ERP29,SEC13,SEC31A,SKP1,TXNDC5,NPLOC4,GANAB Glycolysis / Gluconeogenesis 0.0000141 4.85 PGAM1,ALDOC,DLD,TPI1,ALDH9A1,ALDOB,PGM1,ALDH2,GALM,GPI,ALDH7A1,GAPDH,PGK1,LDHA,ENO1,FBP1,ADH1 Arginine and proline metabolism 0.0000883 4.05 CKB,SRM,ARG1,PYCR3,CNDP2,HOGA1,GOT1,ALDH9A1,ALDH2,OAT,GOT2,LAP3,AGMAT,ALDH7A1 Histidine metabolism 0.00466 2.33
Bi-Allelic GOT2 Mutations Cause a Treatable Malate-Aspartate Shuttle

Please cite this article in press as: van Karnebeek et al., Bi-allelic GOT2 Mutations Cause a Treatable Malate-Aspartate Shuttle-Related Enceph- alopathy, The American Journal of Human Genetics (2019), https://doi.org/10.1016/j.ajhg.2019.07.015 ARTICLE Bi-allelic GOT2 Mutations Cause a Treatable Malate-Aspartate Shuttle-Related Encephalopathy Clara D.M. van Karnebeek,1,2,3,19,21,* Ru´ben J. Ramos,3,4,21 Xiao-Yan Wen,5,6,21 Maja Tarailo-Graovac,7,8,21 Joseph G. Gleeson,9 Cristina Skrypnyk,10 Koroboshka Brand-Arzamendi,5 Farhad Karbassi,5 Mahmoud Y. Issa,11 Robin van der Lee,12 Britt I. Dro¨gemo¨ller,13,14 Janet Koster,15 Justine Rousseau,16 Philippe M. Campeau,16 Youdong Wang,5 Feng Cao,17 Meng Li,5 Jos Ruiter,15 Jolita Ciapaite,3,4 Leo A.J. Kluijtmans,3,18 Michel A.A.P. Willemsen,3,19 Judith J. Jans,3,4 Colin J. Ross,13 Liesbeth T. Wintjes,3,18,20 Richard J. Rodenburg,3,18,19,20 Marleen C.D.G. Huigen,3,18 Zhengping Jia,17 Hans R. Waterham,3,15 Wyeth W. Wasserman,12 Ronald J.A. Wanders,3,15 Nanda M. Verhoeven-Duif,3,4 Maha S. Zaki,11 and Ron A. Wevers3,18,* Early-infantile encephalopathies with epilepsy are devastating conditions mandating an accurate diagnosis to guide proper manage- ment. Whole-exome sequencing was used to investigate the disease etiology in four children from independent families with intellectual disability and epilepsy, revealing bi-allelic GOT2 mutations. In-depth metabolic studies in individual 1 showed low plasma serine, hyper- citrullinemia, hyperlactatemia, and hyperammonemia.