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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2006-08-13 02:23:00 UTC
Update Date2021-09-14 15:45:29 UTC
HMDB IDHMDB0003640
Secondary Accession Numbers
  • HMDB03640
Metabolite Identification
Common NameBeta-Leucine
DescriptionBeta-leucine is a metabolite that is in the middle of a controversy over its presence in the human body. While there are reports that claim it as a human metabolite, there are others that deny its existence. Two examples:. Circulating levels of beta-leucine are elevated in the cobalamin-deficient state of pernicious anemia. Levels of leucine, on the other hand, are much lower. It is proposed that leucine 2,3-aminomutase, the cobalamin-dependent enzyme that catalyzes the interconversion of leucine and beta-leucine, is the affected enzyme in pernicious anemia and causes these results by preventing the synthesis of leucine from beta-leucine. The synthesis of leucine by human leukocytes and hair roots and by rat liver extracts has been shown to occur when either branched chain fatty acids or valine metabolites are the substances. The synthesis is dependent upon adenosylcobalamin and is inhibited by intrinsic factor (PMID:7430116 ). Using forms of beta-leucine and leucine that contain several deuterium atoms in place of several hydrogen atoms as internal standards, techniques have been developed which make it possible to detect and quantitate as little as 0.1 mumol/liter of beta-leucine or leucine in human serum and in incubations containing rat liver supernatant. beta-Leucine was not detectable, i.e. less than 0.1 mumol/liter, in any sera from 50 normal human subjects or in any sera from 50 cobalamin-deficient patients. Experiments in which beta-leucine, leucine, isostearic acid, or isocaproic acid were incubated with rat liver supernatant in the presence or absence of adenosylcobalamin or cobalamin-binding protein failed to demonstrate the formation of leucine or beta-leucine or their interconversion under any of the conditions studied. We conclude that beta-leucine is not present in human blood and that the existence of leucine 2,3-aminomutase in mammalian tissues remains to be established (PMID 3356699 ). Beta-leucine is found to be associated with cobalamin deficiency, which is an inborn error of metabolism.
Structure
Data?1582752284
Synonyms
ValueSource
(S)-Homo-beta-valChEBI
(S)-Homo-b-valGenerator
(S)-Homo-β-valGenerator
b-LeucineGenerator
Β-leucineGenerator
3-Amino-4-methylpentanoic acidMeSH
beta-2-amino-4-Methylvaleric acidHMDB
L-beta-LeucineHMDB
(3S)-b-LeucineGenerator, HMDB
(3S)-Β-leucineGenerator, HMDB
beta-LeucineMeSH
Chemical FormulaC6H13NO2
Average Molecular Weight131.1729
Monoisotopic Molecular Weight131.094628665
IUPAC Name(3S)-3-amino-4-methylpentanoic acid
Traditional Nameβ-leucine
CAS Registry Number5699-54-7
SMILES
CC(C)[C@@H](N)CC(O)=O
InChI Identifier
InChI=1S/C6H13NO2/c1-4(2)5(7)3-6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t5-/m0/s1
InChI KeyGLUJNGJDHCTUJY-YFKPBYRVSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as beta amino acids and derivatives. These are amino acids having a (-NH2) group attached to the beta carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentBeta amino acids and derivatives
Alternative Parents
Substituents
  • Beta amino acid or derivatives
  • Amino fatty acid
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Fatty acyl
  • Fatty acid
  • Amino acid
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Amine
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Hydrocarbon derivative
  • Primary aliphatic amine
  • Organic oxide
  • Carbonyl group
  • Organopnictogen compound
  • Organic oxygen compound
  • Organic nitrogen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Biological location

Source

Route of exposure

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogP0.778Not Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility126 g/LALOGPS
logP10(-2.1) g/LALOGPS
logP10(-1.9) g/LChemAxon
logS10(-0.02) g/LALOGPS
pKa (Strongest Acidic)4.39ChemAxon
pKa (Strongest Basic)10.6ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity34.12 m³·mol⁻¹ChemAxon
Polarizability14.07 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+129.03831661259
DarkChem[M-H]-125.04231661259

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
Beta-LeucineCC(C)[C@@H](N)CC(O)=O2162.1Standard polar33892256
Beta-LeucineCC(C)[C@@H](N)CC(O)=O1148.8Standard non polar33892256
Beta-LeucineCC(C)[C@@H](N)CC(O)=O1192.0Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Beta-Leucine,1TMS,isomer #1CC(C)[C@@H](N)CC(=O)O[Si](C)(C)C1212.1Semi standard non polar33892256
Beta-Leucine,1TMS,isomer #2CC(C)[C@H](CC(=O)O)N[Si](C)(C)C1302.6Semi standard non polar33892256
Beta-Leucine,2TMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C)N[Si](C)(C)C1330.4Semi standard non polar33892256
Beta-Leucine,2TMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C)N[Si](C)(C)C1369.2Standard non polar33892256
Beta-Leucine,2TMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C)N[Si](C)(C)C1470.8Standard polar33892256
Beta-Leucine,2TMS,isomer #2CC(C)[C@H](CC(=O)O)N([Si](C)(C)C)[Si](C)(C)C1517.5Semi standard non polar33892256
Beta-Leucine,2TMS,isomer #2CC(C)[C@H](CC(=O)O)N([Si](C)(C)C)[Si](C)(C)C1431.3Standard non polar33892256
Beta-Leucine,2TMS,isomer #2CC(C)[C@H](CC(=O)O)N([Si](C)(C)C)[Si](C)(C)C1603.6Standard polar33892256
Beta-Leucine,3TMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1543.8Semi standard non polar33892256
Beta-Leucine,3TMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1494.0Standard non polar33892256
Beta-Leucine,3TMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1452.2Standard polar33892256
Beta-Leucine,1TBDMS,isomer #1CC(C)[C@@H](N)CC(=O)O[Si](C)(C)C(C)(C)C1415.0Semi standard non polar33892256
Beta-Leucine,1TBDMS,isomer #2CC(C)[C@H](CC(=O)O)N[Si](C)(C)C(C)(C)C1523.8Semi standard non polar33892256
Beta-Leucine,2TBDMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)N[Si](C)(C)C(C)(C)C1758.9Semi standard non polar33892256
Beta-Leucine,2TBDMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)N[Si](C)(C)C(C)(C)C1788.2Standard non polar33892256
Beta-Leucine,2TBDMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)N[Si](C)(C)C(C)(C)C1756.0Standard polar33892256
Beta-Leucine,2TBDMS,isomer #2CC(C)[C@H](CC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1928.8Semi standard non polar33892256
Beta-Leucine,2TBDMS,isomer #2CC(C)[C@H](CC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1848.3Standard non polar33892256
Beta-Leucine,2TBDMS,isomer #2CC(C)[C@H](CC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1801.8Standard polar33892256
Beta-Leucine,3TBDMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2181.9Semi standard non polar33892256
Beta-Leucine,3TBDMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2126.0Standard non polar33892256
Beta-Leucine,3TBDMS,isomer #1CC(C)[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C1856.9Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Beta-Leucine GC-MS (Non-derivatized) - 70eV, Positivesplash10-000i-9000000000-b4ef4716fcf2c24852fd2017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Beta-Leucine GC-MS (1 TMS) - 70eV, Positivesplash10-00di-9200000000-e6038fb7fedb2b5926222017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Beta-Leucine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Beta-Leucine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine Quattro_QQQ 10V, N/A-QTOF (Annotated)splash10-00di-9200000000-c312d7b4661374b11fe72012-07-25HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine Quattro_QQQ 25V, N/A-QTOF (Annotated)splash10-05fu-9000000000-2f3a81db4c2c8d6143a22012-07-25HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine Quattro_QQQ 40V, N/A-QTOF (Annotated)splash10-0a4l-9000000000-0b2e4113ec38ca7843a12012-07-25HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive-QTOFsplash10-001i-2900000000-5c08508080e2ead24ed12012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive-QTOFsplash10-00di-9100000000-e86097bd64d6da7ab50e2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive-QTOFsplash10-00di-9000000000-8f051218b3e657e92bee2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive-QTOFsplash10-0ab9-9000000000-58d7d5408744e17b8aa92012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive-QTOFsplash10-0a4l-9000000000-918d253495faf90f8c962012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ , positive-QTOFsplash10-001i-2900000000-5c08508080e2ead24ed12017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ , positive-QTOFsplash10-00di-9100000000-e86097bd64d6da7ab50e2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ , positive-QTOFsplash10-00di-9000000000-8f051218b3e657e92bee2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ , positive-QTOFsplash10-0ab9-9000000000-ed73d8f252611f502b472017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Beta-Leucine LC-ESI-QQ , positive-QTOFsplash10-0a4l-9000000000-918d253495faf90f8c962017-09-14HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 10V, Positive-QTOFsplash10-03di-3900000000-1f2691c9c25f658b63fe2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 20V, Positive-QTOFsplash10-0cka-9400000000-c0d4b51d8c4fce50aede2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 40V, Positive-QTOFsplash10-0avl-9000000000-437c8e5e44bc9466d6ed2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 10V, Negative-QTOFsplash10-001i-3900000000-f8aa929f8a383bc99d232017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 20V, Negative-QTOFsplash10-01q0-6900000000-e97f92ca9178bdecdf5c2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 40V, Negative-QTOFsplash10-00y0-9100000000-5664de291dd8fa5bc2772017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 10V, Positive-QTOFsplash10-00di-9200000000-10aae8c247c96ff0e2d72021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 20V, Positive-QTOFsplash10-05fr-9000000000-ffea5f053415eb1d6a602021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 40V, Positive-QTOFsplash10-0a4l-9000000000-410a3f8e8cbee04dd8c72021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 10V, Negative-QTOFsplash10-001i-1900000000-cd68dd10157ab0851fa12021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 20V, Negative-QTOFsplash10-01qm-9300000000-f21cab770afc49f8fa1d2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Beta-Leucine 40V, Negative-QTOFsplash10-0006-9000000000-225d2f3b783518b803302021-09-22Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular LocationsNot Available
Biospecimen Locations
  • Blood
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified4.8 (2.0-8.0) uMAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified24.7 (10.0-50.0) uMAdult (>18 years old)BothCobalamin deficiency details
Associated Disorders and Diseases
Disease References
Vitamin B12 deficiency
  1. Stabler SP, Lindenbaum J, Allen RH: Failure to detect beta-leucine in human blood or leucine 2,3-aminomutase in rat liver using capillary gas chromatography-mass spectrometry. J Biol Chem. 1988 Apr 25;263(12):5581-8. [PubMed:3356699 ]
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB023208
KNApSAcK IDNot Available
Chemspider ID388796
KEGG Compound IDC02486
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkΒ-Leucine
METLIN IDNot Available
PubChem Compound439734
PDB IDNot Available
ChEBI ID86261
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Stabler SP, Lindenbaum J, Allen RH: Failure to detect beta-leucine in human blood or leucine 2,3-aminomutase in rat liver using capillary gas chromatography-mass spectrometry. J Biol Chem. 1988 Apr 25;263(12):5581-8. [PubMed:3356699 ]
  2. Poston JM: Cobalamin-dependent formation of leucine and beta-leucine by rat and human tissue. Changes in pernicious anemia. J Biol Chem. 1980 Nov 10;255(21):10067-72. [PubMed:7430116 ]