DT-Web

DB00167 - L-Isoleucine


Identification
Name L-Isoleucine
Accession Number DB00167 (NUTR00031)
Type small molecule
Description An essential branched-chain aliphatic amino acid found in many proteins. It is an isomer of leucine. It is important in hemoglobin synthesis and regulation of blood sugar and energy levels. [PubChem]
Structure
MOL SDF PDB SMILES InChI
Categories (*)
Molecular Weight 131.1729
Groups approved
Monoisotopic Weight 131.094628665
Pharmacology
Indication The branched-chain amino acids may have antihepatic encephalopathy activity in some. They may also have anticatabolic and antitardive dyskinesia activity.
Mechanism of action (Applies to Valine, Leucine and Isoleucine)
This group of essential amino acids are identified as the branched-chain amino acids, BCAAs. Because this arrangement of carbon atoms cannot be made by humans, these amino acids are an essential element in the diet. The catabolism of all three compounds initiates in muscle and yields NADH and FADH2 which can be utilized for ATP generation. The catabolism of all three of these amino acids uses the same enzymes in the first two steps. The first step in each case is a transamination using a single BCAA aminotransferase, with a-ketoglutarate as amine acceptor. As a result, three different a-keto acids are produced and are oxidized using a common branched-chain a-keto acid dehydrogenase, yielding the three different CoA derivatives. Subsequently the metabolic pathways diverge, producing many intermediates.
The principal product from valine is propionylCoA, the glucogenic precursor of succinyl-CoA. Isoleucine catabolism terminates with production of acetylCoA and propionylCoA; thus isoleucine is both glucogenic and ketogenic. Leucine gives rise to acetylCoA and acetoacetylCoA, and is thus classified as strictly ketogenic.
There are a number of genetic diseases associated with faulty catabolism of the BCAAs. The most common defect is in the branched-chain a-keto acid dehydrogenase. Since there is only one dehydrogenase enzyme for all three amino acids, all three a-keto acids accumulate and are excreted in the urine. The disease is known as Maple syrup urine disease because of the characteristic odor of the urine in afflicted individuals. Mental retardation in these cases is extensive. Unfortunately, since these are essential amino acids, they cannot be heavily restricted in the diet; ultimately, the life of afflicted individuals is short and development is abnormal The main neurological problems are due to poor formation of myelin in the CNS.
Absorption Absorbed from the small intestine by a sodium-dependent active-transport process
Protein binding Not Available
Biotransformation Hepatic
Route of elimination Not Available
Toxicity Symptoms of hypoglycemia, increased mortality in ALS patients taking large doses of BCAAs
Affected organisms
  • Humans and other mammals
Interactions
Drug Interactions Not Available
Food Interactions Not Available

Targets


Isoleucine-tRNA synthetase
Name Isoleucine-tRNA synthetase
Gene Name IARS
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
  • Kedzierski W, Pawelkiewicz J: Interaction of lupin isoleucine-tRNA synthetase with isoleucine transfer ribonucleic acid measured by the thermal inactivation method. Acta Biochim Pol. 1971;18(2):153-61. - Pubmed
  • Schmidt E, Schimmel P: Mutational isolation of a sieve for editing in a transfer RNA synthetase. Science. 1994 Apr 8;264(5156):265-7. - Pubmed
  • Schimmel P, Landro JA, Schmidt E: Evidence for distinct locations for metal binding sites in two closely related class I tRNA synthetases. J Biomol Struct Dyn. 1993 Dec;11(3):571-81. - Pubmed
DTHybrid score 1.0097
Isoleucyl-tRNA synthetase, mitochondrial
Name Isoleucyl-tRNA synthetase, mitochondrial
Gene Name IARS2
Pharmacological action unknown
Actions Not Available
References
  • Wang P, Tang Y, Tirrell DA: Incorporation of trifluoroisoleucine into proteins in vivo. J Am Chem Soc. 2003 Jun 11;125(23):6900-6. - Pubmed
  • Fukunaga R, Fukai S, Ishitani R, Nureki O, Yokoyama S: Crystal structures of the CP1 domain from Thermus thermophilus isoleucyl-tRNA synthetase and its complex with L-valine. J Biol Chem. 2004 Feb 27;279(9):8396-402. Epub 2003 Dec 12. - Pubmed
  • Zhu B, Zhao MW, Eriani G, Wang ED: A present-day aminoacyl-tRNA synthetase with ancestral editing properties. RNA. 2007 Jan;13(1):15-21. Epub 2006 Nov 9. - Pubmed
  • Fukunaga R, Yokoyama S: Crystal structure of leucyl-tRNA synthetase from the archaeon Pyrococcus horikoshii reveals a novel editing domain orientation. J Mol Biol. 2005 Feb 11;346(1):57-71. Epub 2004 Dec 19. - Pubmed
  • Fukunaga R, Yokoyama S: Structural basis for substrate recognition by the editing domain of isoleucyl-tRNA synthetase. J Mol Biol. 2006 Jun 16;359(4):901-12. Epub 2006 Apr 25. - Pubmed
DTHybrid score 0.9881
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Name Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Gene Name ACADSB
Pharmacological action unknown
Actions Not Available
References
  • Korman SH, Andresen BS, Zeharia A, Gutman A, Boneh A, Pitt JJ: 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clin Chem. 2005 Mar;51(3):610-7. Epub 2004 Dec 22. - Pubmed
  • Madsen PP, Kibaek M, Roca X, Sachidanandam R, Krainer AR, Christensen E, Steiner RD, Gibson KM, Corydon TJ, Knudsen I, Wanders RJ, Ruiter JP, Gregersen N, Andresen BS: Short/branched-chain acyl-CoA dehydrogenase deficiency due to an IVS3+3A>G mutation that causes exon skipping. Hum Genet. 2006 Feb;118(6):680-90. Epub 2005 Nov 30. - Pubmed
  • Korman SH: Inborn errors of isoleucine degradation: a review. Mol Genet Metab. 2006 Dec;89(4):289-99. Epub 2006 Sep 6. - Pubmed
DTHybrid score 0.8155
Branched-chain-amino-acid aminotransferase, cytosolic
Name Branched-chain-amino-acid aminotransferase, cytosolic
Gene Name BCAT1
Pharmacological action unknown
Actions Not Available
References
  • Goto M, Miyahara I, Hayashi H, Kagamiyama H, Hirotsu K: Crystal structures of branched-chain amino acid aminotransferase complexed with glutamate and glutarate: true reaction intermediate and double substrate recognition of the enzyme. Biochemistry. 2003 Apr 8;42(13):3725-33. - Pubmed
  • Chen CD, Huang TF, Lin CH, Guan HH, Hsieh YC, Lin YH, Huang YC, Liu MY, Chang WC, Chen CJ: Purification, crystallization and preliminary X-ray crystallographic analysis of branched-chain aminotransferase from Deinococcus radiodurans. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2007 Jun 1;63(Pt 6):492-4. Epub 2007 May 5. - Pubmed
  • Beck HC: Branched-chain fatty acid biosynthesis in a branched-chain amino acid aminotransferase mutant of Staphylococcus carnosus. FEMS Microbiol Lett. 2005 Feb 1;243(1):37-44. - Pubmed
  • Thage BV, Rattray FP, Laustsen MW, Ardo Y, Barkholt V, Houlberg U: Purification and characterization of a branched-chain amino acid aminotransferase from Lactobacillus paracasei subsp. paracasei CHCC 2115. J Appl Microbiol. 2004;96(3):593-602. - Pubmed
  • Madsen SM, Beck HC, Ravn P, Vrang A, Hansen AM, Israelsen H: Cloning and inactivation of a branched-chain-amino-acid aminotransferase gene from Staphylococcus carnosus and characterization of the enzyme. Appl Environ Microbiol. 2002 Aug;68(8):4007-14. - Pubmed
DTHybrid score 0.9293
Branched-chain-amino-acid aminotransferase, mitochondrial
Name Branched-chain-amino-acid aminotransferase, mitochondrial
Gene Name BCAT2
Pharmacological action unknown
Actions Not Available
References
  • Berger BJ, English S, Chan G, Knodel MH: Methionine regeneration and aminotransferases in Bacillus subtilis, Bacillus cereus, and Bacillus anthracis. J Bacteriol. 2003 Apr;185(8):2418-31. - Pubmed
DTHybrid score 0.8539
Isoleucyl-tRNA synthetase, cytoplasmic
Name Isoleucyl-tRNA synthetase, cytoplasmic
Gene Name IARS
Pharmacological action unknown
Actions Not Available
References
  • Crasto CF, Forrest AK, Karoli T, March DR, Mensah L, O'Hanlon PJ, Nairn MR, Oldham MD, Yue W, Banwell MG, Easton CJ: Synthesis and activity of analogues of the isoleucyl tRNA synthetase inhibitor SB-203207. Bioorg Med Chem. 2003 Jul 3;11(13):2687-94. - Pubmed
  • Wang P, Tang Y, Tirrell DA: Incorporation of trifluoroisoleucine into proteins in vivo. J Am Chem Soc. 2003 Jun 11;125(23):6900-6. - Pubmed
  • Mock ML, Michon T, van Hest JC, Tirrell DA: Stereoselective incorporation of an unsaturated isoleucine analogue into a protein expressed in E. coli. Chembiochem. 2006 Jan;7(1):83-7. - Pubmed
  • Fukunaga R, Fukai S, Ishitani R, Nureki O, Yokoyama S: Crystal structures of the CP1 domain from Thermus thermophilus isoleucyl-tRNA synthetase and its complex with L-valine. J Biol Chem. 2004 Feb 27;279(9):8396-402. Epub 2003 Dec 12. - Pubmed
  • Pezo V, Metzgar D, Hendrickson TL, Waas WF, Hazebrouck S, Doring V, Marliere P, Schimmel P, De Crecy-Lagard V: Artificially ambiguous genetic code confers growth yield advantage. Proc Natl Acad Sci U S A. 2004 Jun 8;101(23):8593-7. Epub 2004 May 26. - Pubmed
DTHybrid score 1.0092

Transporters


Monocarboxylate transporter 10
Name Monocarboxylate transporter 10
Gene Name SLC16A10
Actions inhibitor
References
  • Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. - Pubmed
DTHybrid score 0.7379

Predictions


Id Partner name Gene Name Score
246 Valyl-tRNA synthetase VARS 0.0906
501 Leucine carboxyl methyltransferase 1 LCMT1 0.0773
675 Leucine carboxyl methyltransferase 2 LCMT2 0.0773
227 Probable leucyl-tRNA synthetase, mitochondrial LARS2 0.0772
846 Leucyl-tRNA synthetase, cytoplasmic LARS 0.0772
601 Propionyl-CoA carboxylase beta chain, mitochondrial PCCB 0.073
199 Monocarboxylate transporter 8 SLC16A2 0.0681
738 Monocarboxylate transporter 1 SLC16A1 0.0448
245 Large neutral amino acids transporter small subunit 1 SLC7A5 0.0446
1566 Corticoliberin CRH 0.0413
3184 3-methyl-2-oxobutanoate hydroxymethyltransferase panB 0.0406
3315 3-methyl-2-oxobutanoate hydroxymethyltransferase panB 0.0406
3179 2-isopropylmalate synthase leuA 0.0406
301 Kynurenine--oxoglutarate transaminase 1 CCBL1 0.0397
1039 Histone deacetylase 9 HDAC9 0.034
3970 Voltage-dependent N-type calcium channel subunit alpha-1B CACNA1B 0.0339
280 4-aminobutyrate aminotransferase, mitochondrial ABAT 0.0332
489 Monocarboxylate transporter 2 SLC16A7 0.0332
500 Monocarboxylate transporter 4 SLC16A3 0.0332
3334 Deacetoxycephalosporin C synthetase cefE 0.0329
4160 Voltage-dependent calcium channel subunit alpha-2/delta-2 CACNA2D2 0.0318
16 Adenosine A1 receptor ADORA1 0.0297
2744 D-alanine aminotransferase dat 0.0291
2532 Pantoate--beta-alanine ligase panC 0.0285
762 Voltage-dependent calcium channel subunit alpha-2/delta-1 CACNA2D1 0.0274
763 Tyrosine aminotransferase TAT 0.0274
5493 Tyrosine aminotransferase Not Available 0.0274
2746 4-aminobutyrate aminotransferase gabT 0.0255
2749 UDP-4-amino-4-deoxy-L-arabinose--oxoglutarate aminotransferase arnB 0.0251
2842 Glutamate-1-semialdehyde 2,1-aminomutase hemL 0.0251
2249 Aspartate aminotransferase aspC 0.025
2671 Aspartate aminotransferase aspC 0.025
5490 Aspartate aminotransferase Not Available 0.025
6645 D-amino-acid oxidase DAO 0.0245
182 2-oxoglutarate dehydrogenase E1 component, mitochondrial OGDH 0.0244
2546 Histidinol-phosphate aminotransferase hisC 0.0235
2698 Histidinol-phosphate aminotransferase hisC 0.0235
5925 Neuropeptide Y NPY 0.0231
314 System N amino acid transporter 1 SLC38A3 0.0229
534 Threonine synthase-like 1 THNSL1 0.0214
170 Succinate semialdehyde dehydrogenase, mitochondrial ALDH5A1 0.0203
4069 Threonyl-tRNA synthetase, mitochondrial TARS2 0.0197
261 Threonyl-tRNA synthetase, cytoplasmic TARS 0.0197
894 Alanine racemase alr 0.0195
2453 Alanine racemase alr 0.0195
866 Large neutral amino acids transporter small subunit 2 SLC7A8 0.0183
3 Histidine decarboxylase HDC 0.0177
242 Asparagine synthetase [glutamine-hydrolyzing] ASNS 0.0174
6177 UDP-glucuronosyltransferase 1-10 UGT1A10 0.0173
6181 UDP-glucuronosyltransferase 1-8 UGT1A8 0.0171
586 Aspartate aminotransferase, cytoplasmic GOT1 0.0169
5469 Head decoration protein shp 0.0168
349 Serine--pyruvate aminotransferase AGXT 0.0164
3876 Aromatic-L-amino-acid decarboxylase DDC 0.0162
358 Cystathionine beta-synthase CBS 0.0161
2457 Bacillolysin nprS 0.016
3904 Alanine aminotransferase 2 GPT2 0.0152
735 Alanine aminotransferase 1 GPT 0.0152
401 Glutamate [NMDA] receptor subunit zeta-1 GRIN1 0.0151
676 Tyrosine 3-monooxygenase TH 0.0149
6025 UDP-glucuronosyltransferase 1-4 UGT1A4 0.0149
815 Histidyl-tRNA synthetase, cytoplasmic HARS 0.0147
96 Histidine ammonia-lyase HAL 0.0147
6257 Histidine ammonia-lyase Not Available 0.0147
6536 Histidine ammonia-lyase hutH 0.0147
2461 D-alanyl-D-alanine carboxypeptidase Not Available 0.0147
5756 D-alanyl-D-alanine carboxypeptidase dac 0.0147
3877 Growth-inhibiting protein 18 GIG18 0.0146
33 Cystine/glutamate transporter SLC7A11 0.0144
38 Cystinosin CTNS 0.0141
3998 B(0,+)-type amino acid transporter 1 SLC7A9 0.0141
1963 Neutral and basic amino acid transport protein rBAT SLC3A1 0.0141
426 Aspartate aminotransferase, mitochondrial GOT2 0.014
114 Alanine--glyoxylate aminotransferase 2, mitochondrial AGXT2 0.014
169 Cysteine desulfurase, mitochondrial NFS1 0.0138
184 L-serine dehydratase SDS 0.0137
431 Serine racemase SRR 0.0137
150 Cationic amino acid transporter 3 SLC7A3 0.0137
235 High-affinity cationic amino acid transporter 1 SLC7A1 0.0137
58 Cationic amino acid transporter 4 SLC7A4 0.0137
491 Serine palmitoyltransferase 1 SPTLC1 0.0137
727 Serine palmitoyltransferase 2 SPTLC2 0.0137
665 Phosphoserine aminotransferase PSAT1 0.0134
4652 Phosphoserine aminotransferase serC 0.0134
3895 Kynurenine--oxoglutarate transaminase 3 CCBL2 0.0134
801 Kynurenine/alpha-aminoadipate aminotransferase mitochondrial AADAT 0.0134
420 Glutamate decarboxylase 1 GAD1 0.0134
3900 Glutamate decarboxylase 1 GAD1 0.0134
471 Sodium- and chloride-dependent neutral and basic amino acid transporter B(0+) SLC6A14 0.0134
6142 Solute carrier family 22 member 8 SLC22A8 0.0133
118 Organic cation/carnitine transporter 2 SLC22A5 0.013
6176 UDP-glucuronosyltransferase 1-3 UGT1A3 0.013
399 Tyrosyl-tRNA synthetase, mitochondrial YARS2 0.0127
3977 ASRGL1 protein ASRGL1 0.0121
6018 UDP-glucuronosyltransferase 1-9 UGT1A9 0.0121
6106 Cytochrome P450 2C18 CYP2C18 0.0121
6178 UDP-glucuronosyltransferase 2B7 UGT2B7 0.012
263 Neutral amino acid transporter B(0) SLC1A5 0.0119
668 Glutamate--cysteine ligase regulatory subunit GCLM 0.0119
914 Formimidoyltransferase-cyclodeaminase FTCD 0.0115
6143 Solute carrier family 22 member 7 SLC22A7 0.0114
499 Arginine decarboxylase ADC 0.011
221 Lysyl-tRNA synthetase KARS 0.0109
4092 Probable asparaginyl-tRNA synthetase, mitochondrial NARS2 0.0107
669 Asparaginyl-tRNA synthetase, cytoplasmic NARS 0.0107
3899 Alanine--glyoxylate aminotransferase 2-like 2 AGXT2L2 0.0106
186 Glutamate--cysteine ligase catalytic subunit GCLC 0.0103
89 Cysteine sulfinic acid decarboxylase CSAD 0.0102
5650 NimA-related protein DR_0842 0.0101
435 Kynureninase KYNU 0.01
4654 Kynureninase kynU 0.01
72 Seryl-tRNA synthetase, cytoplasmic SARS 0.0099
3984 Probable glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial PET112L 0.0099
355 GMP synthase [glutamine-hydrolyzing] GMPS 0.0099
4353 GMP synthase [glutamine-hydrolyzing] guaA 0.0099
5 Glutaminase liver isoform, mitochondrial GLS2 0.0099
3991 Glutamine-dependent NAD(+) synthetase NADSYN1 0.0099
917 Glutaminase kidney isoform, mitochondrial GLS 0.0099
3982 Phosphoribosylformylglycinamidine synthase PFAS 0.0099
5261 Phosphoribosylformylglycinamidine synthase purL 0.0099
3426 Glutamine synthetase glnA 0.0098
3987 Glutamine synthetase GLUL 0.0098
5718 Cytochrome P450 2A6 CYP2A6 0.0097
42 Phenylalanyl-tRNA synthetase alpha chain FARSA 0.0096
6602 Phenylalanyl-tRNA synthetase alpha chain pheS 0.0096
31 Phenylalanyl-tRNA synthetase, mitochondrial FARS2 0.0096
618 Phenylalanyl-tRNA synthetase beta chain FARSB 0.0096
6603 Phenylalanyl-tRNA synthetase beta chain pheT 0.0096
619 Low-affinity cationic amino acid transporter 2 SLC7A2 0.0095
20 Prostaglandin G/H synthase 1 PTGS1 0.0095
4924 Cytochrome P450 2C8 CYP2C8 0.0094
482 Glycine receptor subunit alpha-1 GLRA1 0.0093
828 Phenylalanine-4-hydroxylase PAH 0.0092
3109 Phenylalanine-4-hydroxylase phhA 0.0092
1729 Solute carrier family 22 member 6 SLC22A6 0.0092
1671 Excitatory amino acid transporter 3 SLC1A1 0.009
3874 Proline synthetase co-transcribed bacterial homolog protein PROSC 0.0089
256 Tyrosyl-tRNA synthetase, cytoplasmic YARS 0.0089
868 Cystathionine gamma-lyase CTH 0.0089
6030 Cytochrome P450 2B6 CYP2B6 0.0087
4025 Mitochondrial glutamate carrier 1 SLC25A22 0.0086
4024 Mitochondrial glutamate carrier 2 SLC25A18 0.0086
4026 Aspartyl aminopeptidase DNPEP 0.0086
664 Glutamate decarboxylase 2 GAD2 0.0086
3890 Glutamate decarboxylase 2 GAD2 0.0086
4023 N-acetylglutamate synthase, mitochondrial NAGS 0.0086
4021 Probable glutamyl-tRNA synthetase, mitochondrial EARS2 0.0086
4022 Glutamate--ammonia ligase domain-containing protein 1 LGSN 0.0086
1912 Excitatory amino acid transporter 1 SLC1A3 0.0086
4016 Excitatory amino acid transporter 5 SLC1A7 0.0086
4019 Excitatory amino acid transporter 4 SLC1A6 0.0086
1047 Excitatory amino acid transporter 2 SLC1A2 0.0086
926 Metabotropic glutamate receptor 7 GRM7 0.0086
919 Glutamate receptor, ionotropic kainate 4 GRIK4 0.0086
915 Delta 1-pyrroline-5-carboxylate synthetase ALDH18A1 0.0086
922 Glutamate receptor 4 GRIA4 0.0086
927 Metabotropic glutamate receptor 8 GRM8 0.0086
925 Metabotropic glutamate receptor 4 GRM4 0.0086
924 Glutamate receptor, ionotropic kainate 3 GRIK3 0.0086
920 Glutamate receptor, ionotropic kainate 5 GRIK5 0.0086
4020 Glutamyl aminopeptidase ENPEP 0.0086
4027 Glutamate receptor delta-1 subunit GRID1 0.0086
4017 5-oxoprolinase OPLAH 0.0086
4018 Glutamate receptor delta-2 subunit GRID2 0.0086
210 Bifunctional aminoacyl-tRNA synthetase [Includes: Glutamyl-tRNA synthetase EPRS 0.0085
4118 Cytochrome P450 3A5 CYP3A5 0.0083
6016 Cytochrome P450 2C19 CYP2C19 0.0079
3254 Bifunctional protein putA [Includes: Proline dehydrogenase putA 0.0077
575 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial GCAT 0.0077
455 5-aminolevulinate synthase, nonspecific, mitochondrial ALAS1 0.0077
681 Glycine dehydrogenase [decarboxylating], mitochondrial GLDC 0.0077
4200 Cytochrome P450 1A2 CYP1A2 0.0077
6152 Solute carrier organic anion transporter family member 2A1 SLCO2A1 0.0076
833 Organic cation/carnitine transporter 1 SLC22A4 0.0075
4757 Cytochrome P450 2C9 CYP2C9 0.0075
505 Glutamate [NMDA] receptor subunit epsilon-3 GRIN2C 0.0073
90 Tryptophan 5-hydroxylase 2 TPH2 0.0073
4063 Tryptophanyl-tRNA synthetase, mitochondrial WARS2 0.0073
830 Glutamate dehydrogenase 2, mitochondrial GLUD2 0.0072
625 Glutamate carboxypeptidase 2 FOLH1 0.0072
3884 SHMT2 protein SHMT2 0.007
3901 SHMT2 protein SHMT2 0.007
3885 Hypothetical protein DKFZp686P09201 DKFZp686P09201 0.007
3879 Serine hydroxymethyltransferase 2 Not Available 0.007
321 Serine hydroxymethyltransferase, mitochondrial SHMT2 0.007
3881 Selenocysteine lyase variant Not Available 0.0069
306 Pyridoxal phosphate phosphatase PDXP 0.0069
3908 P-selectin cytoplasmic tail-associated protein pcap 0.0069
3915 Cysteine sulfinic acid decarboxylase-related protein 1 CSAD 0.0069
3891 Putative L-Dopa decarboxylase DDC 0.0069
3911 Uncharacterized protein C20orf38 SPTLC3 0.0069
3887 Phosphorylase PYGB 0.0069
3906 Serine dehydratase-like SDSL 0.0069
3896 CDNA FLJ46802 fis, clone TRACH3032150, moderately similar to Cysteine sulfinic acid decarboxylase GADL1 0.0069
3914 Alanine-glyoxylate aminotransferase homolog TLH6 0.0069
3909 Hepatic peroxysomal alanine:glyoxylate aminotransferase Not Available 0.0069
3883 Ornithine aminotransferase variant Not Available 0.0069
3880 Serine hydroxymethyltransferase 1 Not Available 0.0069
3893 DDC protein DDC 0.0069
3910 O-phosphoseryl-tRNA(Sec) selenium transferase SEPSECS 0.0069
3878 GAD1 protein GAD1 0.0069
855 Sphingosine-1-phosphate lyase 1 SGPL1 0.0069
3903 Aminolevulinate, delta-, synthase 2 ALAS2 0.0069
3886 Migration-inducing protein 4 ALAS1 0.0069
3894 KIAA0251 protein PDXDC1 0.0069
3875 Glycogen phosphorylase, brain form PYGB 0.0069
3882 Brain glycogen phosphorylase variant Not Available 0.0069
3905 Molybdenum cofactor sulfurase MOCOS 0.0069
591 Glutamate [NMDA] receptor subunit 3B GRIN3B 0.0068
382 Glutamate receptor, ionotropic kainate 1 GRIK1 0.0067
740 Argininosuccinate synthase ASS1 0.0067
865 Argininosuccinate synthase ASS1 0.0067
2680 Argininosuccinate synthase argG 0.0067
3194 Argininosuccinate synthase argG 0.0067
594 Thyroxine-binding globulin SERPINA7 0.0066
916 Metabotropic glutamate receptor 1 GRM1 0.0065
4512 Cytochrome P450 3A4 CYP3A4 0.0065
923 Glutamate receptor 3 GRIA3 0.0063
201 Glutamate dehydrogenase 1, mitochondrial GLUD1 0.0063
837 Glutamate [NMDA] receptor subunit epsilon-1 GRIN2A 0.0063
448 Vitamin K-dependent gamma-carboxylase GGCX 0.0062
83 Alpha-aminoadipic semialdehyde synthase, mitochondrial AASS 0.0062
367 Serine hydroxymethyltransferase, cytosolic SHMT1 0.0062
773 Folylpolyglutamate synthase, mitochondrial FPGS 0.0061
758 Thyroid hormone receptor alpha THRA 0.0061
252 Tryptophanyl-tRNA synthetase, cytoplasmic WARS 0.0059
765 Indoleamine 2,3-dioxygenase IDO1 0.0059
532 Neutral amino acid transporter A SLC1A4 0.0057
4070 Probable alanyl-tRNA synthetase, mitochondrial AARS2 0.0057
323 Alanyl-tRNA synthetase, cytoplasmic AARS 0.0057
3913 Glutamic acid decarboxylase GAD65 0.0057
472 Ornithine aminotransferase, mitochondrial OAT 0.0057
2452 Tryptophanyl-tRNA synthetase trpS 0.0056
439 Glutamate [NMDA] receptor subunit epsilon-4 GRIN2D 0.0056
134 Tryptophan 5-hydroxylase 1 TPH1 0.0055
203 Pyridoxine-5'-phosphate oxidase PNPO 0.0054
346 Thyroid hormone receptor beta-1 THRB 0.0054
6012 Tryptophan 2,3-dioxygenase TDO2 0.0053
171 Cysteine dioxygenase CDO-1 0.0053
774 Cysteinyl-tRNA synthetase, cytoplasmic CARS 0.0052
1314 Thiamine transporter 2 SLC19A3 0.0052
4064 Probable cysteinyl-tRNA synthetase, mitochondrial CARS2 0.0052
705 Glutamate receptor 1 GRIA1 0.0052
207 Glutathione synthetase GSS 0.0052
5269 Glutathione synthetase gshB 0.0052
834 Arginase-2, mitochondrial ARG2 0.0051
464 Glutamate [NMDA] receptor subunit epsilon-2 GRIN2B 0.0051
593 Proton-coupled amino acid transporter 1 SLC36A1 0.0051
6150 Solute carrier organic anion transporter family member 4C1 SLCO4C1 0.0051
6153 Solute carrier organic anion transporter family member 4A1 SLCO4A1 0.005
577 Argininosuccinate lyase ASL 0.0049
3971 CAD protein [Includes: Glutamine-dependent carbamoyl-phosphate synthase CAD 0.0049
449 Ornithine decarboxylase ODC1 0.0048
480 Methionine-R-sulfoxide reductase B2 MSRB2 0.0048
317 Methionine-R-sulfoxide reductase SEPX1 0.0048
3996 Betaine--homocysteine S-methyltransferase 2 BHMT2 0.0048
3921 Methionine adenosyltransferase 2 subunit beta MAT2B 0.0048
3995 Methionyl-tRNA synthetase, mitochondrial MARS2 0.0048
600 Methionyl-tRNA synthetase, cytoplasmic MARS 0.0048
6141 Sodium/bile acid cotransporter SLC10A1 0.0046
6158 Solute carrier organic anion transporter family member 1C1 SLCO1C1 0.0045
1999 Methylated-DNA--protein-cysteine methyltransferase MGMT 0.0043
10 Glycogen phosphorylase, liver form PYGL 0.0043
397 Monocarboxylate transporter 3 SLC16A8 0.0041
218 Monocarboxylate transporter 5 SLC16A4 0.0041
195 Monocarboxylate transporter 7 SLC16A6 0.0041
1632 Solute carrier organic anion transporter family member 2B1 SLCO2B1 0.0041
429 Monocarboxylate transporter 6 SLC16A5 0.0041
191 Peptide methionine sulfoxide reductase MSRA 0.0041
918 Glutamate receptor, ionotropic kainate 2 GRIK2 0.004
706 Glutamate [NMDA] receptor subunit 3A GRIN3A 0.004
373 Transthyretin TTR 0.004
6157 Solute carrier organic anion transporter family member 1B3 SLCO1B3 0.004
626 Cysteine dioxygenase type 1 CDO1 0.004
941 Betaine--homocysteine S-methyltransferase 1 BHMT 0.0039
334 S-adenosylmethionine synthetase isoform type-2 MAT2A 0.0038
921 Glutamate receptor 2 GRIA2 0.0038
453 S-adenosylmethionine synthetase isoform type-1 MAT1A 0.0036
52 Pyruvate kinase isozymes R/L PKLR 0.0035
1490 Solute carrier organic anion transporter family member 1B1 SLCO1B1 0.0035
4085 Peptidyl-prolyl cis-trans isomerase C PPIC 0.0035
4088 Probable prolyl-tRNA synthetase, mitochondrial PARS2 0.0035
4083 Peptidyl-prolyl cis-trans isomerase H PPIH 0.0035
4087 Pyrroline 5-carboxylate reductase isoform P5CR2 0.0035
4090 Chromosome 14 open reading frame 149 C14orf149 0.0035
4082 Proline oxidase, mitochondrial PRODH 0.0035
797 Sodium-dependent proline transporter SLC6A7 0.0035
4086 Peptidyl-prolyl cis-trans isomerase G PPIG 0.0035
6139 Solute carrier organic anion transporter family member 1A2 SLCO1A2 0.0034
110 Pyruvate dehydrogenase E1 component subunit beta, mitochondrial PDHB 0.0034
771 Pyruvate carboxylase, mitochondrial PC 0.0034
1517 Beta-3 adrenergic receptor ADRB3 0.0034
1152 Glycogen phosphorylase, muscle form PYGM 0.0033
840 Methionine synthase reductase, mitochondrial MTRR 0.0033
163 D(1B) dopamine receptor DRD5 0.0032
98 Pyruvate kinase isozymes M1/M2 PKM2 0.0032
2632 Adenylosuccinate synthetase purA 0.0031
3323 Adenylosuccinate synthetase purA 0.0031
3975 Adenylosuccinate synthetase ADSS 0.0031
4598 Adenylosuccinate synthetase Adss 0.0031
6817 Adenylosuccinate synthetase purA 0.0031
3972 Adenylosuccinate synthetase isozyme 2 ADSS 0.0031
533 Aminoacylase-1 ACY1 0.0031
3969 Multifunctional protein ADE2 [Includes: Phosphoribosylaminoimidazole- succinocarboxamide synthase PAICS 0.0031
799 Aspartyl-tRNA synthetase, cytoplasmic DARS 0.0031
209 Aspartoacylase ASPA 0.0031
3979 Aspartoacylase-2 ACY3 0.0031
3976 Aspartyl-tRNA synthetase, mitochondrial DARS2 0.0031
513 Calcium-binding mitochondrial carrier protein Aralar2 SLC25A13 0.0031
305 Mitochondrial aspartate-glutamate carrier protein SLC25A13 0.0031
262 Calcium-binding mitochondrial carrier protein Aralar1 SLC25A12 0.0031
809 Methionine synthase MTR 0.0031
2935 Methionine synthase metH 0.0031
432 D(4) dopamine receptor DRD4 0.003
587 Serum albumin ALB 0.003
4084 Peptidyl-prolyl cis-trans isomerase B PPIB 0.003
638 D(3) dopamine receptor DRD3 0.0029
4000 Prolyl 4-hydroxylase subunit alpha-2 P4HA2 0.0029
7 Nitric oxide synthase, inducible NOS2 0.0029
1008 CTP synthase 1 CTPS 0.0029
3988 Protein-glutamine gamma-glutamyltransferase K TGM1 0.0029
3985 Glucosamine--fructose-6-phosphate aminotransferase [isomerizing] 2 GFPT2 0.0029
3990 Protein-glutamine gamma-glutamyltransferase 4 TGM4 0.0029
3986 Protein-glutamine gamma-glutamyltransferase 6 TGM6 0.0029
3992 Protein-glutamine gamma-glutamyltransferase Z TGM7 0.0029
3983 Protein-glutamine gamma-glutamyltransferase 5 TGM5 0.0029
3989 Glutaminyl-tRNA synthetase QARS 0.0029
291 Nitric-oxide synthase, endothelial NOS3 0.0029
1539 Oligopeptide transporter, small intestine isoform SLC15A1 0.0027
248 Pyrroline-5-carboxylate reductase 1 PYCR1 0.0027
802 Pyrroline-5-carboxylate reductase 2 PYCR2 0.0027
585 Aspartyl/asparaginyl beta-hydroxylase ASPH 0.0027
2554 Peptidyl-prolyl cis-trans isomerase, mitochondrial PPIF 0.0027
2795 Methionine aminopeptidase 2 METAP2 0.0026
23 D(1A) dopamine receptor DRD1 0.0026
3917 Methylenetetrahydrofolate reductase MTHFR 0.0026
789 Alpha-1D adrenergic receptor ADRA1D 0.0026
3949 Prolyl 3-hydroxylase 2 LEPREL1 0.0025
3945 Prolyl 3-hydroxylase 1 LEPRE1 0.0025
3951 Prolyl 3-hydroxylase 3 LEPREL2 0.0025
378 Alpha-2C adrenergic receptor ADRA2C 0.0025
1588 Multidrug resistance protein 1 ABCB1 0.0025
831 D(2) dopamine receptor DRD2 0.0024
1596 Protein-glutamine gamma-glutamyltransferase 2 TGM2 0.0024
629 Alpha-2B adrenergic receptor ADRA2B 0.0024
193 Beta-1 adrenergic receptor ADRB1 0.0024
6 Coagulation factor XIII A chain F13A1 0.0024
67 Prolyl 4-hydroxylase subunit alpha-1 P4HA1 0.0023
766 Beta-2 adrenergic receptor ADRB2 0.0023
1524 Peptidyl-prolyl cis-trans isomerase A PPIA 0.0023
6700 Peptidyl-prolyl cis-trans isomerase A ppiA 0.0023
632 Alpha-1B adrenergic receptor ADRA1B 0.0023
540 Sodium-dependent noradrenaline transporter SLC6A2 0.0023
413 Amidophosphoribosyltransferase PPAT 0.0023
2515 Amidophosphoribosyltransferase purF 0.0023
3714 Amidophosphoribosyltransferase purF 0.0023
4058 Glycine N-acyltransferase-like protein 2 GLYATL2 0.0022
4057 Glycine N-acyltransferase GLYAT 0.0022
4059 Glycine N-acyltransferase-like protein 1 GLYATL1 0.0022
4056 N-arachidonyl glycine receptor GPR18 0.0022
4061 Peroxisomal sarcosine oxidase PIPOX 0.0022
4055 Bile acid CoA:amino acid N-acyltransferase BAAT 0.0022
1236 Sodium- and chloride-dependent glycine transporter 1 SLC6A9 0.0022
4062 Sodium- and chloride-dependent glycine transporter 2 SLC6A5 0.0022
4060 Vesicular inhibitory amino acid transporter SLC32A1 0.0022
1088 5-aminolevulinate synthase, erythroid-specific, mitochondrial ALAS2 0.0022
1466 Glycyl-tRNA synthetase GARS 0.0022
318 Alpha-2A adrenergic receptor ADRA2A 0.0022
3978 Adenylosuccinate synthetase isozyme 1 ADSSL1 0.0021
556 Alpha-1A adrenergic receptor ADRA1A 0.002
2339 Protein-glutamine gamma-glutamyltransferase E TGM3 0.002
4119 Cytochrome P450 2D6 CYP2D6 0.0018
4054 Glycine cleavage system H protein, mitochondrial GCSH 0.0017
62 Glycine receptor subunit beta GLRB 0.0017
383 Glycine amidinotransferase, mitochondrial GATM 0.0016
411 Glycine N-methyltransferase GNMT 0.0016
5682 Ribonuclease pancreatic RNASE1 0.0016
820 Glycine receptor subunit alpha-2 GLRA2 0.0014
461 Glycine receptor subunit alpha-3 GLRA3 0.0014
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