Showing Compound Card for L-Isoleucine (CDB000132)

Jump To Section:
IdentificationTaxonomyOntologyPhysical propertiesSpectraProtein targetPathwaysConcentrationsLinksReferencesenzymes (6)transporters (1) Show 7 proteinsXML
Record Information
Version1.0
Created at2020-03-18 23:25:58 UTC
Updated at2022-12-13 23:36:23 UTC
CannabisDB IDCDB000132
Secondary Accession NumbersNot Available
Cannabis Compound Identification
Common NameL-Isoleucine
DescriptionL-isoleucine or isoleucine, abbreviated as Ile or I, is one of the essential amino acids used in the biosynthesis of proteins. It contains an α-amino group (which is in the protonated −NH3+ form under biological conditions), an α-carboxylic acid group (which is in the deprotonated −COO− form under biological conditions), and a hydrocarbon side chain with a branch (a central carbon atom bound to three other carbon atoms). It is classified as a non-polar, uncharged (at physiological pH), branched-chain, aliphatic amino acid. In plants and microorganisms, it is synthesized via several steps, starting from pyruvate and alpha-ketobutyrate. Isoleucine is one of the three branched chain amino acids (BCAA), together with leucine and valine. Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. Isoleucine is less concentrated in foods compared to leucine and valine. A cup of milk contains 800 mg of leucine and only 500 mg of isoleucine and valine. A cup of wheat germ has about 1.6 g of leucine and 1 g of isoleucine and valine. Eggs and cheese have about equal amounts of the BCAA and contain about 400 mg of leucine and 400 mg of valine and isoleucine. Pork contains 7 to 8 g of leucine and 3 to 4 g of isoleucine and valine. L-isoleucine is also found in Cannabis plants (PMID: 6991645 ). These BCAA are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine, and isoleucine, respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. BCAA are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia or portacaval shunt; aromatic amino acids (AAA)-tyrosine, tryptophan and phenylalanine, as well as methionine-are increased in these conditions. Valine has been established as a useful supplemental therapy to the ailing liver. All the BCAA probably compete with AAA for absorption into the brain. Supplemental BCAA with vitamin B6 and zinc help normalize the BCAA:AAA ratio. The BCAA are not without side effects. Leucine alone, for example, exacerbates pellagra and can cause psychosis in pellagra patients by increasing excretion of niacin in the urine. Leucine may lower brain serotonin and dopamine whereas isoleucine may have potential as an antipsychotic treatment. A dose of 3 g of isoleucine added to the niacin regime cleared leucine-aggravated psychosis in schizophrenic patients. Moreover, L-isoleucine is associated with maple syrup urine disease (MSUD; PMID: 10234605 ), which is an inborn error of metabolism, so named for the sweet, maple syrup smell of urine. MSUD is an autosomal recessive disorder that affects the metabolism of the BCAA. Mutations occur in the 4 genes that comprise the branched-chain alpha-keto acid dehydrogenase complex, which breaks down the BCAA.
Structure
Thumb
MOLSDF3D-SDFPDBSMILESInChI View 3D Structure
Synonyms
ValueSource
(2S,3S)-2-Amino-3-methylpentanoic acidChEBI
2-Amino-3-methylvaleric acidChEBI
alpha-Amino-beta-methylvaleric acidChEBI
IChEBI
IleChEBI
ISOLEUCINEChEBI
(2S,3S)-2-Amino-3-methylpentanoateGenerator
2-Amino-3-methylvalerateGenerator
a-Amino-b-methylvalerateGenerator
a-Amino-b-methylvaleric acidGenerator
alpha-Amino-beta-methylvalerateGenerator
Α-amino-β-methylvalerateGenerator
Α-amino-β-methylvaleric acidGenerator
(2S,3S)-2-Amino-3-methyl-pentanoateHMDB
(2S,3S)-2-Amino-3-methyl-pentanoic acidHMDB
(2S,3S)-a-Amino-b-methyl-N-valerateHMDB
(2S,3S)-a-Amino-b-methyl-N-valeric acidHMDB
(2S,3S)-a-Amino-b-methylvalerateHMDB
(2S,3S)-a-Amino-b-methylvaleric acidHMDB
(2S,3S)-Alph-amino-beta-methylvalerateHMDB
(2S,3S)-Alph-amino-beta-methylvaleric acidHMDB
(2S,3S)-alpha-Amino-beta-merthyl-N-valerateHMDB
(2S,3S)-alpha-Amino-beta-merthyl-N-valeric acidHMDB
(2S,3S)-alpha-Amino-beta-merthylvalerateHMDB
(2S,3S)-alpha-Amino-beta-merthylvaleric acidHMDB
(2S,3S)-alpha-Amino-beta-methyl-N-valerateHMDB
(2S,3S)-alpha-Amino-beta-methyl-N-valeric acidHMDB
(2S,3S)-alpha-Amino-beta-methylvalerateHMDB
(2S,3S)-alpha-Amino-beta-methylvaleric acidHMDB
(S)-IsoleucineHMDB
(S,S)-IsoleucineHMDB
2-Amino-3-methylpentanoateHMDB
2-Amino-3-methylpentanoic acidHMDB
2S,3S-IsoleucineHMDB
Erythro-L-isoleucineHMDB
Iso-leucineHMDB
L-(+)-IsoleucineHMDB
L-IleHMDB
[S-(R*,r*)]-2-amino-3-methylpentanoateHMDB
[S-(R*,r*)]-2-amino-3-methylpentanoic acidHMDB
Isoleucine, L-isomerHMDB
AlloisoleucineHMDB
Isoleucine, L isomerHMDB
L-Isomer isoleucineHMDB
2S-Amino-3S-methylpentanoateHMDB
L-IsoleucineKEGG
Chemical FormulaC6H13NO2
Average Molecular Weight131.17
Monoisotopic Molecular Weight131.0946
IUPAC Name(2S,3S)-2-amino-3-methylpentanoic acid
Traditional NameL-isoleucine
CAS Registry Number73-32-5
SMILES
CC[C@H](C)[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1
InChI KeyAGPKZVBTJJNPAG-WHFBIAKZSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as isoleucine and derivatives. Isoleucine and derivatives are compounds containing isoleucine or a derivative thereof resulting from reaction of isoleucine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentIsoleucine and derivatives
Alternative Parents
Substituents
  • Isoleucine or derivatives
  • Alpha-amino acid
  • L-alpha-amino acid
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Fatty acid
  • Fatty acyl
  • Amino acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxide
  • Organopnictogen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Organic oxygen compound
  • Amine
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point285.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility35 mg/mLHuman Metabolome Project
logP-1.70HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
logP-1.7ALOGPS
logP-1.5ChemAxon
logS-0.06ALOGPS
pKa (Strongest Acidic)2.79ChemAxon
pKa (Strongest Basic)9.59ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity34.09 m³·mol⁻¹ChemAxon
Polarizability14.11 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
EI-MS/GC-MS
TypeDescriptionSplash KeyView
EI-MSMass Spectrum (Electron Ionization)splash10-004r-9000000000-34d4d4cb7042da231eb42014-09-20View Spectrum
GC-MSL-Isoleucine, 2 TMS, GC-MS Spectrumsplash10-0a4i-0930000000-e78f845bb2a8d4736476Spectrum
GC-MSL-Isoleucine, 2 TMS, GC-MS Spectrumsplash10-0a4i-0910000000-de9162d149073d0e2a37Spectrum
GC-MSL-Isoleucine, 2 TMS, GC-MS Spectrumsplash10-0a4i-0920000000-599e61f8ccdb6525c7a9Spectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-0a4i-0910000000-742e44c426c0d6c2a9acSpectrum
GC-MSL-Isoleucine, 2 TMS, GC-MS Spectrumsplash10-05fr-8910000000-dbb33e0f02ac2ca5feddSpectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-0a4i-0920000000-37690f426455ac41e2c2Spectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-0a4i-0930000000-e78f845bb2a8d4736476Spectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-0a4i-0910000000-de9162d149073d0e2a37Spectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-0a4i-0920000000-599e61f8ccdb6525c7a9Spectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-0a4i-0910000000-742e44c426c0d6c2a9acSpectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-0udi-2392000000-74cca1eb265b8e3d7f43Spectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-05fr-8910000000-dbb33e0f02ac2ca5feddSpectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-000i-9100000000-27891dff695c5db53796Spectrum
GC-MSL-Isoleucine, non-derivatized, GC-MS Spectrumsplash10-0a4i-0910000000-43ec1890f6dbd6aea657Spectrum
Predicted GC-MSL-Isoleucine, non-derivatized, Predicted GC-MS Spectrum - 70eV, Positivesplash10-00b9-9100000000-40bc9ef43da5f18be883Spectrum
Predicted GC-MSL-Isoleucine, 1 TMS, Predicted GC-MS Spectrum - 70eV, Positivesplash10-000i-9200000000-ae8694c0f3e4dcc608f9Spectrum
Predicted GC-MSL-Isoleucine, non-derivatized, Predicted GC-MS Spectrum - 70eV, PositiveNot AvailableSpectrum
Predicted GC-MSL-Isoleucine, TMS_1_2, Predicted GC-MS Spectrum - 70eV, PositiveNot AvailableSpectrum
Predicted GC-MSL-Isoleucine, TBDMS_1_1, Predicted GC-MS Spectrum - 70eV, PositiveNot AvailableSpectrum
Predicted GC-MSL-Isoleucine, TBDMS_1_2, Predicted GC-MS Spectrum - 70eV, PositiveNot AvailableSpectrum
MS/MS
TypeDescriptionSplash KeyView
MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-9300000000-8a23b3e62231eb65f80f2012-07-24View Spectrum
MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00ko-9000000000-ac7e71578d7e1d0e8ce92012-07-24View Spectrum
MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-052f-9000000000-2586d4a089921dd977ed2012-07-24View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-5b11521ff6a631376d2b2012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-3c352f229e4067fcd4892012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-8a7ef48fc0c1b6f845c22012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0002-0920000000-2e11aa1c7a5defc386db2012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-b63ac9cf06eda4c54a812012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-e5b0c8c6b09f541d6dbf2012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-d89d16d5c2242e44ec632012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-5de583142c7bdb381c8f2012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-001i-0900000000-8c75bde35c4a4073ee652012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-001i-0900000000-233a9862f616afea2d172012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-001i-1900000000-375f35065b82b13e6d6e2012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-00dl-9000000000-d0d4a9f90fe2aca744832012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0006-9000000000-0018f47571feaf232ff82012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-001r-7900000000-278dc67396114be331ba2012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-000i-9000000000-e26c042aa6231eeca0712012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-014r-9000000000-b6c1752fd3fbccb3d1c52012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-05mo-9000000000-0569c3162621252ed0a82012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-052f-9000000000-3d52b5d56d3fab45276e2012-08-31View Spectrum
MS/MSLC-MS/MS Spectrum - CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positivesplash10-001i-0900000000-720554d58264a9cfdb672012-08-31View Spectrum
Predicted MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0019-9500000000-00268b2694cde281641b2016-09-12View Spectrum
Predicted MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-9000000000-4014793e17e0290790a42016-09-12View Spectrum
Predicted MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4i-9000000000-eb9a97a14461e524e76e2016-09-12View Spectrum
NMR
TypeDescriptionView
1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, experimental)Spectrum
1D NMR13C NMR Spectrum (1D, 125 MHz, H2O, experimental)Spectrum
1D NMR13C NMR Spectrum (1D, 22.5 MHz, D2O, experimental)Spectrum
1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)Spectrum
1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)Spectrum
1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)Spectrum
1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)Spectrum
1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)Spectrum
1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)Spectrum
1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)Spectrum
1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)Spectrum
1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)Spectrum
1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)Spectrum
1D NMR13C NMR Spectrum (1D, 400 MHz, H2O, experimental)Spectrum
2D NMR[1H, 1H]-TOCSY. Unexported temporarily by An Chi on Oct 15, 2021 until json or nmrML file is generated. 2D NMR Spectrum (experimental)Spectrum
2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)Spectrum
Pathways
Pathways
Protein Targets
Enzymes
Protein NameGene NameLocusUniprot IDDetails
Branched-chain-amino-acid aminotransferase, cytosolicBCAT112p12.1P54687 details
Branched-chain-amino-acid aminotransferase, mitochondrialBCAT219q13O15382 details
Isoleucine--tRNA ligase, cytoplasmicIARS9q21P41252 details
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrialACADSB10q26.13P45954 details
L-amino-acid oxidaseIL4I119q13.3-q13.4Q96RQ9 details
Isoleucine--tRNA ligase, mitochondrialIARS21q41Q9NSE4 details
Transporters
Protein NameGene NameLocusUniprot IDDetails
Monocarboxylate transporter 10SLC16A106q21-q22Q8TF71 details
Metal BindingsNot Available
ReceptorsNot Available
Transcriptional FactorsNot Available
Concentrations Data
Cannabis CultivarStatusValueReferenceDetails
Alien DawgDetected and Quantified0.416 mg/g dry wt
    • David S. Wishart,...
 details
GabriolaDetected and Quantified0.191 mg/g dry wt
    • David S. Wishart,...
 details
Island HoneyDetected and Quantified0.429 mg/g dry wt
    • David S. Wishart,...
 details
QuadraDetected and Quantified0.331 mg/g dry wt
    • David S. Wishart,...
 details
Sensi StarDetected and Quantified0.0613 mg/g dry wt
    • David S. Wishart,...
 details
Tangerine DreamDetected and Quantified0.120 mg/g dry wt
    • David S. Wishart,...
 details
HMDB IDHMDB0000172
DrugBank IDDB00167
Phenol Explorer Compound IDNot Available
FoodDB IDFDB012397
KNApSAcK IDC00001374
Chemspider ID6067
KEGG Compound IDC00407
BioCyc IDILE
BiGG ID34887
Wikipedia LinkIsoleucine
METLIN ID5193
PubChem Compound6306
PDB IDNot Available
ChEBI ID17191
References
General References
  1. Turner CE, Elsohly MA, Boeren EG: Constituents of Cannabis sativa L. XVII. A review of the natural constituents. J Nat Prod. 1980 Mar-Apr;43(2):169-234. doi: 10.1021/np50008a001. [PubMed:6991645 ]
  2. Podebrad F, Heil M, Reichert S, Mosandl A, Sewell AC, Bohles H: 4,5-dimethyl-3-hydroxy-2[5H]-furanone (sotolone)--the odour of maple syrup urine disease. J Inherit Metab Dis. 1999 Apr;22(2):107-14. doi: 10.1023/a:1005433516026. [PubMed:10234605 ]

Enzymes

Enzyme Details
1. Branched-chain-amino-acid aminotransferase, cytosolic
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:
BCAT1
Uniprot ID:
P54687
Molecular weight:
38644.77
Enzyme Details
2. Branched-chain-amino-acid aminotransferase, mitochondrial
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids.
Gene Name:
BCAT2
Uniprot ID:
O15382
Molecular weight:
33776.315
Enzyme Details
3. Isoleucine--tRNA ligase, cytoplasmic
General function:
Involved in nucleotide binding
Specific function:
Not Available
Gene Name:
IARS
Uniprot ID:
P41252
Molecular weight:
144496.915
Enzyme Details
4. Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
General function:
Involved in acyl-CoA dehydrogenase activity
Specific function:
Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
Gene Name:
ACADSB
Uniprot ID:
P45954
Molecular weight:
47485.035
Enzyme Details
5. L-amino-acid oxidase
General function:
Involved in oxidoreductase activity
Specific function:
Lysosomal L-amino-acid oxidase with highest specific activity with phenylalanine. May play a role in lysosomal antigen processing and presentation (By similarity).
Gene Name:
IL4I1
Uniprot ID:
Q96RQ9
Molecular weight:
65327.26
Enzyme Details
6. Isoleucine--tRNA ligase, mitochondrial
General function:
Involved in nucleotide binding
Specific function:
Not Available
Gene Name:
IARS2
Uniprot ID:
Q9NSE4
Molecular weight:
113790.565

Transporters

Enzyme Details
1. Monocarboxylate transporter 10
General function:
Involved in transmembrane transport
Specific function:
Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:
SLC16A10
Uniprot ID:
Q8TF71
Molecular weight:
55492.1