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Identification
NameL-Isoleucine
Accession NumberDB00167  (NUTR00031)
TypeSmall Molecule
GroupsApproved, Nutraceutical
DescriptionAn 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
Thumb
Synonyms
(2S,3S)-2-Amino-3-methylpentanoic acid
2-Amino-3-methylvaleric acid
alpha-amino-beta-methylvaleric acid
I
Ile
Isoleucine
L-Isoleucine
α-amino-β-methylvaleric acid
External Identifiers Not Available
Approved Prescription ProductsNot Available
Approved Generic Prescription ProductsNot Available
Approved Over the Counter ProductsNot Available
Unapproved/Other Products Not Available
International BrandsNot Available
Brand mixtures
NameLabellerIngredients
2.5% Travasol Amino Acid Injection With Electrolytes In 10% DextroseBaxter Corporation Clintec Nutrition Division
2.5% Travasol Amino Acid Injection With Electrolytes In 10% Dextrose ClinimixBaxter Corporation Clintec Nutrition Division
2.5% Travasol Amino Acid Injection Without Electrolytes In 10% Dextrose QuickmixBaxter Corporation Clintec Nutrition Division
2.5%travasol Amino Acid InJ.W.eleC.W.25%dexClintec Nutrition Company
2.75% Travas. Amino Acid InJ.W.elecw.25%dexClintec Nutrition Company
2.75% Travasol Amino Acid Injection With Electrolytes In 25% Dextrose QuickmixBaxter Corporation Clintec Nutrition Division
2.75% Travasol Amino Acid Injection With Electrolytes In 5% Dextrose QuickmixBaxter Corporation Clintec Nutrition Division
2.75%travasol Amino Acid InJ.W.eleC.W.5%dex.Clintec Nutrition Company
4.25% Amino Acid Injection Without Electrolytes In 20% Dextrose QuickmixBaxter Corporation Clintec Nutrition Division
4.25% Travasol Amino Acid Injection With Electrolytes In 10% DextroseBaxter Corporation Clintec Nutrition Division
4.25% Travasol Amino Acid Injection With Electrolytes In 20% DextroseBaxter Corporation Clintec Nutrition Division
4.25% Travasol Amino Acid Injection With Electrolytes In 20% Dextrose QuickmixBaxter Corporation Clintec Nutrition Division
4.25% Travasol Amino Acid Injection With Electrolytes In 25% DextroseBaxter Corporation Clintec Nutrition Division
4.25% Travasol Amino Acid Injection With Electrolytes In 5% Dextrose QuickmixBaxter Corporation Clintec Nutrition Division
4.25% Travasol Amino Acid Injection Without Electrlytes In 10% DextroseBaxter Corporation Clintec Nutrition Division
4.25% Travasol Amino Acid Injection Without Electrolytes In 20% DextroseBaxter Corporation Clintec Nutrition Division
4.25% Travasol Amino Acid Injection Without Electrolytes In 25% DextroseBaxter Corporation Clintec Nutrition Division
4.25% Travasol Amino Acid Without Electrolytes In 5% Dextrose QuickmixBaxter Corporation Clintec Nutrition Division
4.25%trav. Amino Acid InJ.W.O.elect.5%dext.Clintec Nutrition Company
4.25%travasol Amino Acid InJ.W.elecw.5%dex.Clintec Nutrition Company
5% Travasol Amino Acid Injection With Electrolytes In 20% DextroseBaxter Corporation Clintec Nutrition Division
5% Travasol Amino Acid Injection Without Electrolytes In 20% DextroseBaxter Corporation Clintec Nutrition Division
5% Travasol Amino Acid Injection Without Electrolytes In 25% DextroseBaxter Corporation Clintec Nutrition Division
AminosynHospira, Inc.
Aminosyn 10%Hospira Healthcare Corporation
Aminosyn 10% W ElectrolytesAbbott Laboratories, Limited
Aminosyn 3.5% MAbbott Laboratories, Limited
Aminosyn 5%Hospira Healthcare Corporation
Aminosyn 7%Hospira Healthcare Corporation
Aminosyn 8.5%Hospira Healthcare Corporation
Aminosyn 8.5% Injection With ElectrolytesHospira Healthcare Corporation
Aminosyn IIHospira, Inc.
Aminosyn II 10%Hospira Healthcare Corporation
Aminosyn II 10% With ElectrolytesHospira Healthcare Corporation
Aminosyn II 15%Hospira Healthcare Corporation
Aminosyn II 5% InjAbbott Laboratories, Limited
Aminosyn II 7% InjectionHospira Healthcare Corporation
Aminosyn II 7% M In 10% Dextrose(dual Chamber)Hospira Healthcare Corporation
Aminosyn II 7% With 10% DextroseHospira Healthcare Corporation
Aminosyn II 7% With 50% DextroseHospira Healthcare Corporation
Aminosyn II 8.5% InjectionHospira Healthcare Corporation
Aminosyn II 8.5% M In 20% Dextrose (dual Chamber)Hospira Healthcare Corporation
Aminosyn II 8.5% With 50% DextroseHospira Healthcare Corporation
Aminosyn II In Dextrose InjectionHospira Healthcare Corporation
Aminosyn II With ElectrolytesHospira, Inc.
Aminosyn RFHospira, Inc.
Aminosyn RF InjectionHospira Healthcare Corporation
Aminosyn Sulfite FreeHospira, Inc.
Aminosyn-PFHospira, Inc.
Aminosyn-PF 10%Hospira Healthcare Corporation
Aminosyn-PF 7%Hospira Healthcare Corporation
Bcaa 4% InjBaxter Corporation
ClinimixBaxter Corporation
Clinimix 2.5% Travasol Aa Without Electrolytes In 10% Dextrose InjecBaxter Corporation Clintec Nutrition Division
Clinimix EBaxter Corporation
ClinisolBaxter Healthcare Corporation
Freamine HbcB. Braun Medical Inc.
Freamine IIIB. Braun Medical Inc.
HepatamineB. Braun Medical Inc.
NephramineB. Braun Medical Inc.
NovamineHospira, Inc.
Olimel 3.3% EBaxter Corporation
Olimel 4.4%Baxter Corporation
Olimel 4.4% EBaxter Corporation
Olimel 5.7%Baxter Corporation
Olimel 5.7% EBaxter Corporation
Periolimel 2.5% EBaxter Corporation
PlenamineB. Braun Medical Inc.
Premasol - Sulfite-free (amino Acid)Baxter Healthcare Corporation
Primene 10%Baxter Corporation
Primene 10%-liq IVClintec Nutrition Company
ProsolBaxter Healthcare Corporation
Quick Mix 2.5% Travasol Aa With Electrolytes With 25% Dextrose InjecBaxter Corporation Clintec Nutrition Division
Renamin (amino Acids) InjectionBaxter Corporation
TravasolBaxter Healthcare Corporation
Travasol Amino Acid Inj 5.5%Baxter Corporation
Travasol Amino Acid Inj 8.5%Baxter Corporation
Travasol EBaxter Corporation
Travasol Inj Without Electrolytes 5.5%Baxter Corporation
TrophamineB. Braun Medical Inc.
Vamin 18 Electrolyte-freeFresenius Kabi Ab
Vamin NFresenius Kabi Ab
SaltsNot Available
Categories
UNII04Y7590D77
CAS number73-32-5
WeightAverage: 131.1729
Monoisotopic: 131.094628665
Chemical FormulaC6H13NO2
InChI KeyAGPKZVBTJJNPAG-WHFBIAKZSA-N
InChI
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
IUPAC Name
(2S,3S)-2-amino-3-methylpentanoic acid
SMILES
CC[[email protected]](C)[[email protected]](N)C(O)=O
Pharmacology
IndicationThe branched-chain amino acids may have antihepatic encephalopathy activity in some. They may also have anticatabolic and antitardive dyskinesia activity.
Structured Indications Not Available
PharmacodynamicsThey provide ingredients for the manufacturing of other essential biochemical components in the body, some of which are utilized for the production of energy, stimulants to the upper brain and helping you to be more alert.
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.
TargetKindPharmacological actionActionsOrganismUniProt ID
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrialProteinunknownNot AvailableHumanP45954 details
Branched-chain-amino-acid aminotransferase, cytosolicProteinunknownNot AvailableHumanP54687 details
Branched-chain-amino-acid aminotransferase, mitochondrialProteinunknownNot AvailableHumanO15382 details
Isoleucine--tRNA ligase, cytoplasmicProteinunknownNot AvailableHumanP41252 details
Isoleucine--tRNA ligase, mitochondrialProteinunknownNot AvailableHumanQ9NSE4 details
Related Articles
AbsorptionAbsorbed from the small intestine by a sodium-dependent active-transport process
Volume of distributionNot Available
Protein bindingNot Available
Metabolism

Hepatic

Route of eliminationNot Available
Half lifeNot Available
ClearanceNot Available
ToxicitySymptoms of hypoglycemia, increased mortality in ALS patients taking large doses of BCAAs
Affected organisms
  • Humans and other mammals
Pathways
PathwayCategorySMPDB ID
Valine, Leucine and Isoleucine DegradationMetabolicSMP00032
Beta-Ketothiolase DeficiencyDiseaseSMP00173
Amikacin Action PathwayDrug actionSMP00253
Spectinomycin Action PathwayDrug actionSMP00258
3-Methylcrotonyl Coa Carboxylase Deficiency Type IDiseaseSMP00237
Clindamycin Action PathwayDrug actionSMP00249
Gentamicin Action PathwayDrug actionSMP00254
Netilmicin Action PathwayDrug actionSMP00257
Doxycycline Action PathwayDrug actionSMP00291
Minocycline Action PathwayDrug actionSMP00292
Lymecycline Action PathwayDrug actionSMP00295
Tigecycline Action PathwayDrug actionSMP00712
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencyDiseaseSMP00137
3-Methylglutaconic Aciduria Type IDiseaseSMP00139
3-Methylglutaconic Aciduria Type IVDiseaseSMP00141
Maple Syrup Urine DiseaseDiseaseSMP00199
Propionic AcidemiaDiseaseSMP00236
Tobramycin Action PathwayDrug actionSMP00711
Arbekacin Action PathwayDrug actionSMP00713
Methacycline Action PathwayDrug actionSMP00727
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencyDiseaseSMP00138
Azithromycin Action PathwayDrug actionSMP00247
Telithromycin Action PathwayDrug actionSMP00252
Streptomycin Action PathwayDrug actionSMP00259
Demeclocycline Action PathwayDrug actionSMP00290
Oxytetracycline Action PathwayDrug actionSMP00293
Tetracycline Action PathwayDrug actionSMP00294
3-hydroxyisobutyric acid dehydrogenase deficiencyDiseaseSMP00521
3-hydroxyisobutyric aciduriaDiseaseSMP00522
Lincomycin Action PathwayDrug actionSMP00728
SNP Mediated EffectsNot Available
SNP Mediated Adverse Drug ReactionsNot Available
Interactions
Drug InteractionsNot Available
Food InteractionsNot Available
References
Synthesis Reference

Nelli I. Zhdanova, Tatyana V. Leonova, Ljudmila F. Kozyreva, “Method of producing L-isoleucine using Brevibacterium flavum.” U.S. Patent US4237228, issued February, 1962.

US4237228
General ReferencesNot Available
External Links
ATC CodesNot Available
AHFS CodesNot Available
PDB Entries
FDA labelNot Available
MSDSDownload (72.8 KB)
ADMET
Predicted ADMET features
PropertyValueProbability
Human Intestinal Absorption+0.9677
Blood Brain Barrier+0.78
Caco-2 permeable-0.7966
P-glycoprotein substrateNon-substrate0.7385
P-glycoprotein inhibitor INon-inhibitor0.9825
P-glycoprotein inhibitor IINon-inhibitor0.9739
Renal organic cation transporterNon-inhibitor0.9696
CYP450 2C9 substrateNon-substrate0.8513
CYP450 2D6 substrateNon-substrate0.8372
CYP450 3A4 substrateNon-substrate0.7827
CYP450 1A2 substrateNon-inhibitor0.8536
CYP450 2C9 inhibitorNon-inhibitor0.8762
CYP450 2D6 inhibitorNon-inhibitor0.9
CYP450 2C19 inhibitorNon-inhibitor0.9386
CYP450 3A4 inhibitorNon-inhibitor0.9155
CYP450 inhibitory promiscuityLow CYP Inhibitory Promiscuity0.97
Ames testNon AMES toxic0.903
CarcinogenicityNon-carcinogens0.632
BiodegradationReady biodegradable0.5166
Rat acute toxicity1.5846 LD50, mol/kg Not applicable
hERG inhibition (predictor I)Weak inhibitor0.9921
hERG inhibition (predictor II)Non-inhibitor0.9735
ADMET data is predicted using admetSAR, a free tool for evaluating chemical ADMET properties. (23092397 )
Pharmacoeconomics
ManufacturersNot Available
Packagers
Dosage forms
FormRouteStrength
LiquidIntravenous
Injection, solutionIntravenous
Injection, solution, concentrateIntravenous
EmulsionIntravenous
SolutionIntravenous
InjectionIntravenous
Prices
Unit descriptionCostUnit
L-isoleucine powder3.21USD g
DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.
PatentsNot Available
Properties
StateSolid
Experimental Properties
PropertyValueSource
melting point285.5 dec °CPhysProp
water solubility3.44E+004 mg/L (at 25 °C)YALKOWSKY,SH & DANNENFELSER,RM (1992)
logP-1.70HANSCH,C ET AL. (1995)
pKa2.37 (at 0 °C)KORTUM,G ET AL (1961)
Predicted Properties
PropertyValueSource
Water Solubility114.0 mg/mLALOGPS
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 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity34.09 m3·mol-1ChemAxon
Polarizability14.11 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Mass Spec (NIST)Download (2.96 KB)
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0a4i-0930000000-e78f845bb2a8d4736476View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0a4i-0910000000-de9162d149073d0e2a37View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0a4i-0920000000-599e61f8ccdb6525c7a9View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-0a4i-0910000000-742e44c426c0d6c2a9acView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-05fr-8910000000-dbb33e0f02ac2ca5feddView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MSNot Available
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-9300000000-8a23b3e62231eb65f80fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00ko-9000000000-ac7e71578d7e1d0e8ce9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-052f-9000000000-2586d4a089921dd977edView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-5b11521ff6a631376d2bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-3c352f229e4067fcd489View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-8a7ef48fc0c1b6f845c2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0002-0920000000-2e11aa1c7a5defc386dbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-b63ac9cf06eda4c54a81View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-e5b0c8c6b09f541d6dbfView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-d89d16d5c2242e44ec63View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-5de583142c7bdb381c8fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-001i-0900000000-8c75bde35c4a4073ee65View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-001i-0900000000-233a9862f616afea2d17View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-001i-1900000000-375f35065b82b13e6d6eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-00dl-9000000000-d0d4a9f90fe2aca74483View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0006-9000000000-0018f47571feaf232ff8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-001r-7900000000-278dc67396114be331baView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-000i-9000000000-e26c042aa6231eeca071View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-014r-9000000000-b6c1752fd3fbccb3d1c5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-05mo-9000000000-0569c3162621252ed0a8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-052f-9000000000-3d52b5d56d3fab45276eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positivesplash10-001i-0900000000-720554d58264a9cfdb67View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, PositiveNot Available
MSMass Spectrum (Electron Ionization)splash10-004r-9000000000-34d4d4cb7042da231eb4View in MoNA
1D NMR1H NMR SpectrumNot Available
1D NMR13C NMR SpectrumNot Available
1D NMR13C NMR SpectrumNot Available
2D NMR[1H,1H] 2D NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
Substituents
  • L-alpha-amino acid
  • Methyl-branched fatty acid
  • Branched fatty acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors

Targets

Kind
Protein
Organism
Human
Pharmacological action
unknown
General Function:
Oxidoreductase activity, acting on the ch-ch group of donors, with a flavin as acceptor
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 Da
References
  1. 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:15615815 ]
  2. 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:16317551 ]
  3. Korman SH: Inborn errors of isoleucine degradation: a review. Mol Genet Metab. 2006 Dec;89(4):289-99. Epub 2006 Sep 6. [PubMed:16950638 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
General Function:
L-valine transaminase 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:
42965.815 Da
References
  1. 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:12667063 ]
  2. 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:17554170 ]
  3. 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:15667998 ]
  4. 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:14962140 ]
  5. 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:12147502 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
General Function:
L-valine transaminase 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:
44287.445 Da
References
  1. 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:12670965 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
General Function:
Isoleucine-trna ligase activity
Specific Function:
Not Available
Gene Name:
IARS
Uniprot ID:
P41252
Molecular Weight:
144496.915 Da
References
  1. 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:12788342 ]
  2. 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:12783542 ]
  3. 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:16397872 ]
  4. 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:14672940 ]
  5. 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:15163798 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
General Function:
Isoleucine-trna ligase activity
Specific Function:
Not Available
Gene Name:
IARS2
Uniprot ID:
Q9NSE4
Molecular Weight:
113790.565 Da
References
  1. 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:12783542 ]
  2. 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:14672940 ]
  3. 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:17095543 ]
  4. 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:15663927 ]
  5. 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:16697013 ]

Transporters

Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
inhibitor
General Function:
Transporter activity
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 (By similarity).
Gene Name:
SLC16A10
Uniprot ID:
Q8TF71
Molecular Weight:
55492.07 Da
References
  1. 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:11278508 ]
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Drug created on June 13, 2005 07:24 / Updated on August 17, 2016 12:23