DT-Web

DB00129 - L-Ornithine


Identification
Name L-Ornithine
Accession Number DB00129 (NUTR00043)
Type small molecule
Description Ornithine is an amino acid produced in the urea cycle by the splitting off of urea from arginine. It is a central part of the urea cycle, which allows for the disposal of excess nitrogen. L-Ornithine is a precursor of citrulline and arginine.
Structure
MOL SDF PDB SMILES InChI
Categories (*)
Molecular Weight 132.161
Groups approved
Monoisotopic Weight 132.089877638
Pharmacology
Indication Used for nutritional supplementation, also for treating dietary shortage or imbalance. It has been claimed that ornithine improves athletic performance, has anabolic effects, has wound-healing effects, and is immuno-enhancing.
Mechanism of action L-Ornithine is metabolised to L-arginine. L-arginine stimulates the pituitary release of growth hormone. Burns or other injuries affect the state of L-arginine in tissues throughout the body. As De novo synthesis of L-arginine during these conditions is usually not sufficient for normal immune function, nor for normal protein synthesis, L-ornithine may have immunomodulatory and wound-healing activities under these conditions (by virtue of its metabolism to L-arginine).
Absorption Absorbed from the small intestine via a sodium-dependent active transport process
Protein binding Not Available
Biotransformation Ornithine undergoes extensive metabolism in the liver to L-arginine, polyamines, and proline, and several other metabolites.
Route of elimination Not Available
Toxicity Oral, rat LD50 = 10000 mg/kg
Affected organisms
  • Humans and other mammals
Interactions
Drug Interactions Not Available
Food Interactions Not Available

Targets


Ornithine aminotransferase, mitochondrial
Name Ornithine aminotransferase, mitochondrial
Gene Name OAT
Pharmacological action unknown
Actions Not Available
References
  • Levillain O, Balvay S, Peyrol S: Localization and differential expression of arginase II in the kidney of male and female mice. Pflugers Arch. 2005 Feb;449(5):491-503. Epub 2004 Dec 23. - Pubmed
  • Inubushi T, Takasawa T, Tuboi Y, Watanabe N, Aki K, Katunuma N: Changes of glucose metabolism and skin-collagen neogenesis in vitamin B6 deficiency. Biofactors. 2005;23(2):59-67. - Pubmed
  • Kaneko S, Ueda-Yamada M, Ando A, Matsumura S, Okuda-Ashitaka E, Matsumura M, Uyama M, Ito S: Cytotoxic effect of spermine on retinal pigment epithelial cells. Invest Ophthalmol Vis Sci. 2007 Jan;48(1):455-63. - Pubmed
DTHybrid score 1.2051
Ornithine carbamoyltransferase, mitochondrial
Name Ornithine carbamoyltransferase, mitochondrial
Gene Name OTC
Pharmacological action unknown
Actions Not Available
References
  • Barcelona-Andres B, Marina A, Rubio V: Gene structure, organization, expression, and potential regulatory mechanisms of arginine catabolism in Enterococcus faecalis. J Bacteriol. 2002 Nov;184(22):6289-300. - Pubmed
  • El Alami M, Dubois E, Oudjama Y, Tricot C, Wouters J, Stalon V, Messenguy F: Yeast epiarginase regulation, an enzyme-enzyme activity control: identification of residues of ornithine carbamoyltransferase and arginase responsible for enzyme catalytic and regulatory activities. J Biol Chem. 2003 Jun 13;278(24):21550-8. Epub 2003 Apr 4. - Pubmed
  • Templeton MD, Reinhardt LA, Collyer CA, Mitchell RE, Cleland WW: Kinetic analysis of the L-ornithine transcarbamoylase from Pseudomonas savastanoi pv. phaseolicola that is resistant to the transition state analogue (R)-N delta-(N'-sulfodiaminophosphinyl)-L-ornithine. Biochemistry. 2005 Mar 22;44(11):4408-15. - Pubmed
  • Morizono H, Cabrera-Luque J, Shi D, Gallegos R, Yamaguchi S, Yu X, Allewell NM, Malamy MH, Tuchman M: Acetylornithine transcarbamylase: a novel enzyme in arginine biosynthesis. J Bacteriol. 2006 Apr;188(8):2974-82. - Pubmed
DTHybrid score 1.7553
Arginase-1
Name Arginase-1
Gene Name ARG1
Pharmacological action unknown
Actions Not Available
References
  • Gobert AP, Cheng Y, Wang JY, Boucher JL, Iyer RK, Cederbaum SD, Casero RA Jr, Newton JC, Wilson KT: Helicobacter pylori induces macrophage apoptosis by activation of arginase II. J Immunol. 2002 May 1;168(9):4692-700. - Pubmed
  • Iwata S, Tsujino T, Ikeda Y, Ishida T, Ueyama T, Gotoh T, Mori M, Yokoyama M: Decreased expression of arginase II in the kidneys of Dahl salt-sensitive rats. Hypertens Res. 2002 May;25(3):411-8. - Pubmed
  • Stickings P, Mistry SK, Boucher JL, Morris SM, Cunningham JM: Arginase expression and modulation of IL-1beta-induced nitric oxide generation in rat and human islets of Langerhans. Nitric Oxide. 2002 Dec;7(4):289-96. - Pubmed
  • Bansal V, Ochoa JB: Arginine availability, arginase, and the immune response. Curr Opin Clin Nutr Metab Care. 2003 Mar;6(2):223-8. - Pubmed
  • El Alami M, Dubois E, Oudjama Y, Tricot C, Wouters J, Stalon V, Messenguy F: Yeast epiarginase regulation, an enzyme-enzyme activity control: identification of residues of ornithine carbamoyltransferase and arginase responsible for enzyme catalytic and regulatory activities. J Biol Chem. 2003 Jun 13;278(24):21550-8. Epub 2003 Apr 4. - Pubmed
DTHybrid score 1.9344
Ornithine decarboxylase antizyme
Name Ornithine decarboxylase antizyme
Gene Name OAZ1
Pharmacological action unknown
Actions Not Available
References
  • Lopez-Contreras AJ, Lopez-Garcia C, Jimenez-Cervantes C, Cremades A, Penafiel R: Mouse ornithine decarboxylase-like gene encodes an antizyme inhibitor devoid of ornithine and arginine decarboxylating activity. J Biol Chem. 2006 Oct 13;281(41):30896-906. Epub 2006 Aug 17. - Pubmed
  • Yamaguchi Y, Takatsuka Y, Matsufuji S, Murakami Y, Kamio Y: Characterization of a counterpart to Mammalian ornithine decarboxylase antizyme in prokaryotes. J Biol Chem. 2006 Feb 17;281(7):3995-4001. Epub 2005 Dec 14. - Pubmed
  • Janne J, Alhonen L, Pietila M, Keinanen TA: Genetic approaches to the cellular functions of polyamines in mammals. Eur J Biochem. 2004 Mar;271(5):877-94. - Pubmed
DTHybrid score 0.9807
Low-affinity cationic amino acid transporter 2
Name Low-affinity cationic amino acid transporter 2
Gene Name SLC7A2
Pharmacological action unknown
Actions Not Available
References
  • Yeramian A, Martin L, Arpa L, Bertran J, Soler C, McLeod C, Modolell M, Palacin M, Lloberas J, Celada A: Macrophages require distinct arginine catabolism and transport systems for proliferation and for activation. Eur J Immunol. 2006 Jun;36(6):1516-26. - Pubmed
  • Cerec V, Piquet-Pellorce C, Aly HA, Touzalin AM, Jegou B, Bauche F: Multiple pathways for cationic amino Acid transport in rat seminiferous tubule cells. Biol Reprod. 2007 Feb;76(2):241-9. Epub 2006 Oct 25. - Pubmed
  • Endo M, Oyadomari S, Terasaki Y, Takeya M, Suga M, Mori M, Gotoh T: Induction of arginase I and II in bleomycin-induced fibrosis of mouse lung. Am J Physiol Lung Cell Mol Physiol. 2003 Aug;285(2):L313-21. Epub 2003 Apr 4. - Pubmed
DTHybrid score 1.1888
Mitochondrial ornithine transporter 2
Name Mitochondrial ornithine transporter 2
Gene Name SLC25A2
Pharmacological action unknown
Actions Not Available
References
  • Camacho JA, Rioseco-Camacho N, Andrade D, Porter J, Kong J: Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder. Mol Genet Metab. 2003 Aug;79(4):257-71. - Pubmed
  • Begum L, Jalil MA, Kobayashi K, Iijima M, Li MX, Yasuda T, Horiuchi M, del Arco A, Satrustegui J, Saheki T: Expression of three mitochondrial solute carriers, citrin, aralar1 and ornithine transporter, in relation to urea cycle in mice. Biochim Biophys Acta. 2002 Apr 12;1574(3):283-92. - Pubmed
  • Camacho JA, Mardach R, Rioseco-Camacho N, Ruiz-Pesini E, Derbeneva O, Andrade D, Zaldivar F, Qu Y, Cederbaum SD: Clinical and functional characterization of a human ORNT1 mutation (T32R) in the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. Pediatr Res. 2006 Oct;60(4):423-9. Epub 2006 Aug 28. - Pubmed
DTHybrid score 0.9923
Cationic amino acid transporter 4
Name Cationic amino acid transporter 4
Gene Name SLC7A4
Pharmacological action unknown
Actions Not Available
References
  • Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. - Pubmed
  • Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. - Pubmed
DTHybrid score 1.2418
High-affinity cationic amino acid transporter 1
Name High-affinity cationic amino acid transporter 1
Gene Name SLC7A1
Pharmacological action unknown
Actions Not Available
References
  • Rotmann A, Strand D, Martine U, Closs EI: Protein kinase C activation promotes the internalization of the human cationic amino acid transporter hCAT-1. A new regulatory mechanism for hCAT-1 activity. J Biol Chem. 2004 Dec 24;279(52):54185-92. Epub 2004 Oct 15. - Pubmed
  • Cerec V, Piquet-Pellorce C, Aly HA, Touzalin AM, Jegou B, Bauche F: Multiple pathways for cationic amino Acid transport in rat seminiferous tubule cells. Biol Reprod. 2007 Feb;76(2):241-9. Epub 2006 Oct 25. - Pubmed
  • Yeramian A, Martin L, Arpa L, Bertran J, Soler C, McLeod C, Modolell M, Palacin M, Lloberas J, Celada A: Macrophages require distinct arginine catabolism and transport systems for proliferation and for activation. Eur J Immunol. 2006 Jun;36(6):1516-26. - Pubmed
DTHybrid score 1.2525
Arginase-2, mitochondrial
Name Arginase-2, mitochondrial
Gene Name ARG2
Pharmacological action unknown
Actions Not Available
References
  • Rodriguez PC, Zea AH, DeSalvo J, Culotta KS, Zabaleta J, Quiceno DG, Ochoa JB, Ochoa AC: L-arginine consumption by macrophages modulates the expression of CD3 zeta chain in T lymphocytes. J Immunol. 2003 Aug 1;171(3):1232-9. - Pubmed
  • Levillain O, Hus-Citharel A, Garvi S, Peyrol S, Reymond I, Mutin M, Morel F: Ornithine metabolism in male and female rat kidney: mitochondrial expression of ornithine aminotransferase and arginase II. Am J Physiol Renal Physiol. 2004 Apr;286(4):F727-38. Epub 2004 Feb 10. - Pubmed
  • Levillain O, Diaz JJ, Blanchard O, Dechaud H: Testosterone down-regulates ornithine aminotransferase gene and up-regulates arginase II and ornithine decarboxylase genes for polyamines synthesis in the murine kidney. Endocrinology. 2005 Feb;146(2):950-9. Epub 2004 Nov 11. - Pubmed
  • Bussiere FI, Chaturvedi R, Cheng Y, Gobert AP, Asim M, Blumberg DR, Xu H, Kim PY, Hacker A, Casero RA Jr, Wilson KT: Spermine causes loss of innate immune response to Helicobacter pylori by inhibition of inducible nitric-oxide synthase translation. J Biol Chem. 2005 Jan 28;280(4):2409-12. Epub 2004 Nov 17. - Pubmed
  • Nissim I, Luhovyy B, Horyn O, Daikhin Y, Nissim I, Yudkoff M: The role of mitochondrially bound arginase in the regulation of urea synthesis: studies with [U-15N4]arginine, isolated mitochondria, and perfused rat liver. J Biol Chem. 2005 May 6;280(18):17715-24. Epub 2005 Mar 7. - Pubmed
DTHybrid score 1.2335
Mitochondrial ornithine transporter 1
Name Mitochondrial ornithine transporter 1
Gene Name SLC25A15
Pharmacological action unknown
Actions Not Available
References
  • Korman SH, Kanazawa N, Abu-Libdeh B, Gutman A, Tsujino S: Hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome with evidence of mitochondrial dysfunction due to a novel SLC25A15 (ORNT1) gene mutation in a Palestinian family. J Neurol Sci. 2004 Mar 15;218(1-2):53-8. - Pubmed
  • Camacho JA, Mardach R, Rioseco-Camacho N, Ruiz-Pesini E, Derbeneva O, Andrade D, Zaldivar F, Qu Y, Cederbaum SD: Clinical and functional characterization of a human ORNT1 mutation (T32R) in the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. Pediatr Res. 2006 Oct;60(4):423-9. Epub 2006 Aug 28. - Pubmed
DTHybrid score 0.9922
Cationic amino acid transporter 3
Name Cationic amino acid transporter 3
Gene Name SLC7A3
Pharmacological action unknown
Actions Not Available
References
  • Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. - Pubmed
  • Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. - Pubmed
  • Hosokawa H, Sawamura T, Kobayashi S, Ninomiya H, Miwa S, Masaki T: Cloning and characterization of a brain-specific cationic amino acid transporter. J Biol Chem. 1997 Mar 28;272(13):8717-22. - Pubmed
DTHybrid score 1.2505
Ornithine decarboxylase antizyme 2
Name Ornithine decarboxylase antizyme 2
Gene Name OAZ2
Pharmacological action unknown
Actions Not Available
References
  • Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. - Pubmed
  • Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. - Pubmed
DTHybrid score 0.9806
Glycine amidinotransferase, mitochondrial
Name Glycine amidinotransferase, mitochondrial
Gene Name GATM
Pharmacological action unknown
Actions Not Available
References
  • Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. - Pubmed
  • Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. - Pubmed
  • Thomasset N, Quash GA, Dore JF: The differential contribution of arginase and transamidinase to ornithine biosynthesis in two achromic human melanoma cell lines. FEBS Lett. 1982 Nov 1;148(1):63-6. - Pubmed
  • Natesan S, Reddy SR: Compensatory changes in enzymes of arginine metabolism during renal hypertrophy in mice. Comp Biochem Physiol B Biochem Mol Biol. 2001 Dec;130(4):585-95. - Pubmed
  • Mitruka BM, Costilow RN: Arginine and ornithine catabolism by Clostridium botulinum. J Bacteriol. 1967 Jan;93(1):295-301. - Pubmed
DTHybrid score 1.4587
Ornithine decarboxylase antizyme 3
Name Ornithine decarboxylase antizyme 3
Gene Name OAZ3
Pharmacological action unknown
Actions Not Available
References
  • Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. - Pubmed
  • Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. - Pubmed
DTHybrid score 0.9789

Predictions


Id Partner name Gene Name Score
221 Lysyl-tRNA synthetase KARS 0.4023
6151 Monocarboxylate transporter 10 SLC16A10 0.2598
833 Organic cation/carnitine transporter 1 SLC22A4 0.2278
2842 Glutamate-1-semialdehyde 2,1-aminomutase hemL 0.2082
740 Argininosuccinate synthase ASS1 0.1577
865 Argininosuccinate synthase ASS1 0.1577
2680 Argininosuccinate synthase argG 0.1577
3194 Argininosuccinate synthase argG 0.1577
499 Arginine decarboxylase ADC 0.1564
577 Argininosuccinate lyase ASL 0.1464
56 Delta-aminolevulinic acid dehydratase ALAD 0.1186
2356 Delta-aminolevulinic acid dehydratase hemB 0.1186
3613 Delta-aminolevulinic acid dehydratase hemB 0.1186
3674 Delta-aminolevulinic acid dehydratase hemB 0.1186
7 Nitric oxide synthase, inducible NOS2 0.1122
291 Nitric-oxide synthase, endothelial NOS3 0.1119
4551 Lantibiotic mersacidin mrsA 0.0738
1278 Pro-epidermal growth factor EGF 0.0682
118 Organic cation/carnitine transporter 2 SLC22A5 0.0673
864 Gag-Pol polyprotein gag-pol 0.059
2237 Gag-Pol polyprotein gag-pol 0.059
2326 Gag-Pol polyprotein gag-pol 0.059
2451 Gag-Pol polyprotein gag-pol 0.059
2901 Gag-Pol polyprotein gag-pol 0.059
3165 Gag-Pol polyprotein gag-pol 0.059
3722 Gag-Pol polyprotein gag-pol 0.059
6306 Gag-Pol polyprotein gag-pol 0.059
6624 Gag-Pol polyprotein gag-pol 0.059
76 Nitric-oxide synthase, brain NOS1 0.0394
3627 UPF0190 protein yedY yedY 0.0376
1392 Catenin beta-1 CTNNB1 0.0375
3352 Structural polyprotein Not Available 0.0375
3628 Structural polyprotein Not Available 0.0375
4892 Structural polyprotein Not Available 0.0375
2617 Nitric oxide synthase oxygenase nos 0.0369
2701 Nitric oxide synthase oxygenase nos 0.0369
843 NG,NG-dimethylarginine dimethylaminohydrolase 1 DDAH1 0.0319
670 NG,NG-dimethylarginine dimethylaminohydrolase 2 DDAH2 0.0319
4066 Protein-arginine deiminase type-1 PADI1 0.0319
4067 Protein-arginine deiminase type-3 PADI3 0.0319
4068 Protein-arginine deiminase type-2 PADI2 0.0319
4065 Protein-arginine deiminase type-6 PADI6 0.0319
1814 Protein-arginine deiminase type-4 PADI4 0.0275
365 Dihydrofolate reductase DHFR 0.0255
2381 Dihydrofolate reductase DFR1 0.0255
2833 Dihydrofolate reductase Not Available 0.0255
2931 Dihydrofolate reductase folA 0.0255
3544 Dihydrofolate reductase folA 0.0255
3682 Dihydrofolate reductase folA 0.0255
6642 Dihydrofolate reductase folA 0.0255
6756 Dihydrofolate reductase dfrA 0.0255
5691 DTDP-4-DEHYDRORHAMNOSE 3,5-EPIMERASE RMLC rmlC 0.0152
5694 Transcription antiterminator licT licT 0.0152
3361 Serpin B5 SERPINB5 0.0152
357 Carbonic anhydrase 2 CA2 0.0139
575 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial GCAT 0.012
455 5-aminolevulinate synthase, nonspecific, mitochondrial ALAS1 0.0119
681 Glycine dehydrogenase [decarboxylating], mitochondrial GLDC 0.0119
2286 Isocitrate dehydrogenase [NADP] icd 0.0115
2302 Isocitrate dehydrogenase [NADP] icd 0.0115
2771 Isocitrate dehydrogenase [NADP] icd 0.0115
4534 Cytohesin-2 CYTH2 0.0109
3884 SHMT2 protein SHMT2 0.0107
3901 SHMT2 protein SHMT2 0.0107
3885 Hypothetical protein DKFZp686P09201 DKFZp686P09201 0.0107
3879 Serine hydroxymethyltransferase 2 Not Available 0.0107
321 Serine hydroxymethyltransferase, mitochondrial SHMT2 0.0107
114 Alanine--glyoxylate aminotransferase 2, mitochondrial AGXT2 0.0106
349 Serine--pyruvate aminotransferase AGXT 0.0106
3668 Maltose-binding periplasmic protein precursor malE 0.0105
367 Serine hydroxymethyltransferase, cytosolic SHMT1 0.01
4058 Glycine N-acyltransferase-like protein 2 GLYATL2 0.0096
4059 Glycine N-acyltransferase-like protein 1 GLYATL1 0.0096
1088 5-aminolevulinate synthase, erythroid-specific, mitochondrial ALAS2 0.0096
4061 Peroxisomal sarcosine oxidase PIPOX 0.0096
4055 Bile acid CoA:amino acid N-acyltransferase BAAT 0.0096
1466 Glycyl-tRNA synthetase GARS 0.0096
4060 Vesicular inhibitory amino acid transporter SLC32A1 0.0096
4057 Glycine N-acyltransferase GLYAT 0.0096
4056 N-arachidonyl glycine receptor GPR18 0.0096
1236 Sodium- and chloride-dependent glycine transporter 1 SLC6A9 0.0096
4062 Sodium- and chloride-dependent glycine transporter 2 SLC6A5 0.0096
2592 Beta-galactosidase lacZ 0.0093
5692 Calcium/calmodulin-dependent 3',5'-cyclic nucleotide phosphodiesterase 1B PDE1B 0.0082
4054 Glycine cleavage system H protein, mitochondrial GCSH 0.0081
2347 Proto-oncogene serine/threonine-protein kinase Pim-1 PIM1 0.008
411 Glycine N-methyltransferase GNMT 0.0072
62 Glycine receptor subunit beta GLRB 0.0071
6263 cAMP-dependent protein kinase catalytic subunit alpha PRKACA 0.0071
207 Glutathione synthetase GSS 0.0069
5269 Glutathione synthetase gshB 0.0069
24 Thymidylate synthase TMP1 0.0066
359 Thymidylate synthase TYMS 0.0066
2626 Thymidylate synthase thyA 0.0066
2729 Thymidylate synthase thyA 0.0066
5352 Thymidylate synthase THYA 0.0066
3883 Ornithine aminotransferase variant Not Available 0.0066
593 Proton-coupled amino acid transporter 1 SLC36A1 0.0065
541 cAMP-specific 3',5'-cyclic phosphodiesterase 4B PDE4B 0.0063
505 Glutamate [NMDA] receptor subunit epsilon-3 GRIN2C 0.0062
820 Glycine receptor subunit alpha-2 GLRA2 0.0057
591 Glutamate [NMDA] receptor subunit 3B GRIN3B 0.0057
461 Glycine receptor subunit alpha-3 GLRA3 0.0057
3908 P-selectin cytoplasmic tail-associated protein pcap 0.0055
3915 Cysteine sulfinic acid decarboxylase-related protein 1 CSAD 0.0055
306 Pyridoxal phosphate phosphatase PDXP 0.0055
3911 Uncharacterized protein C20orf38 SPTLC3 0.0055
3906 Serine dehydratase-like SDSL 0.0055
3896 CDNA FLJ46802 fis, clone TRACH3032150, moderately similar to Cysteine sulfinic acid decarboxylase GADL1 0.0055
3891 Putative L-Dopa decarboxylase DDC 0.0055
3909 Hepatic peroxysomal alanine:glyoxylate aminotransferase Not Available 0.0055
3881 Selenocysteine lyase variant Not Available 0.0055
3878 GAD1 protein GAD1 0.0055
3887 Phosphorylase PYGB 0.0055
3914 Alanine-glyoxylate aminotransferase homolog TLH6 0.0055
3893 DDC protein DDC 0.0055
3880 Serine hydroxymethyltransferase 1 Not Available 0.0055
3910 O-phosphoseryl-tRNA(Sec) selenium transferase SEPSECS 0.0055
3875 Glycogen phosphorylase, brain form PYGB 0.0055
3882 Brain glycogen phosphorylase variant Not Available 0.0055
855 Sphingosine-1-phosphate lyase 1 SGPL1 0.0055
3905 Molybdenum cofactor sulfurase MOCOS 0.0055
3903 Aminolevulinate, delta-, synthase 2 ALAS2 0.0055
664 Glutamate decarboxylase 2 GAD2 0.0055
3890 Glutamate decarboxylase 2 GAD2 0.0055
3886 Migration-inducing protein 4 ALAS1 0.0055
3894 KIAA0251 protein PDXDC1 0.0055
837 Glutamate [NMDA] receptor subunit epsilon-1 GRIN2A 0.0052
482 Glycine receptor subunit alpha-1 GLRA1 0.0049
534 Threonine synthase-like 1 THNSL1 0.0049
3899 Alanine--glyoxylate aminotransferase 2-like 2 AGXT2L2 0.0049
3874 Proline synthetase co-transcribed bacterial homolog protein PROSC 0.0049
431 Serine racemase SRR 0.0048
184 L-serine dehydratase SDS 0.0048
491 Serine palmitoyltransferase 1 SPTLC1 0.0048
89 Cysteine sulfinic acid decarboxylase CSAD 0.0048
727 Serine palmitoyltransferase 2 SPTLC2 0.0048
665 Phosphoserine aminotransferase PSAT1 0.0048
4652 Phosphoserine aminotransferase serC 0.0048
801 Kynurenine/alpha-aminoadipate aminotransferase mitochondrial AADAT 0.0048
3895 Kynurenine--oxoglutarate transaminase 3 CCBL2 0.0048
420 Glutamate decarboxylase 1 GAD1 0.0048
3900 Glutamate decarboxylase 1 GAD1 0.0048
3 Histidine decarboxylase HDC 0.0047
435 Kynureninase KYNU 0.0047
4654 Kynureninase kynU 0.0047
203 Pyridoxine-5'-phosphate oxidase PNPO 0.0047
3913 Glutamic acid decarboxylase GAD65 0.0047
169 Cysteine desulfurase, mitochondrial NFS1 0.0045
868 Cystathionine gamma-lyase CTH 0.0044
301 Kynurenine--oxoglutarate transaminase 1 CCBL1 0.0044
3877 Growth-inhibiting protein 18 GIG18 0.0044
426 Aspartate aminotransferase, mitochondrial GOT2 0.0044
358 Cystathionine beta-synthase CBS 0.0043
914 Formimidoyltransferase-cyclodeaminase FTCD 0.0043
763 Tyrosine aminotransferase TAT 0.0042
5493 Tyrosine aminotransferase Not Available 0.0042
735 Alanine aminotransferase 1 GPT 0.0042
3904 Alanine aminotransferase 2 GPT2 0.0042
449 Ornithine decarboxylase ODC1 0.0042
795 Branched-chain-amino-acid aminotransferase, mitochondrial BCAT2 0.0041
586 Aspartate aminotransferase, cytoplasmic GOT1 0.0041
574 Branched-chain-amino-acid aminotransferase, cytosolic BCAT1 0.0038
10 Glycogen phosphorylase, liver form PYGL 0.0037
280 4-aminobutyrate aminotransferase, mitochondrial ABAT 0.0036
3876 Aromatic-L-amino-acid decarboxylase DDC 0.0035
1152 Glycogen phosphorylase, muscle form PYGM 0.0029
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