L-Alloisoleucine

L-Alloisoleucine Description

L-alloisoleucine is a branched chain amino acid and is a stereo-isomer of L-isoleucine. It is a common constituent of human plasma (albeit at low levels). L-alloisoleucine is produced as a byproduct of isoleucine transamination. L-Isoleucine is one of the four common amino acids (the 3 others being threonine, hydroxyproline, and hydroxylysine) that have two asymmetric carbon atoms which produce four structural possibilities for the same chemical composition. L-alloisoleucine differs from L-isoleucine by having having different stereochemistry around its beta carbon. Plasma L-alloisoleucine, which is derived from L-isoleucine in vivo, can be used for the diagnosis of maple syrup urine disease (MSUD). Indeed, plasma L-alloisoleucine levels above 5 umol/L is the most specific and most sensitive diagnostic marker for all forms of MSUD (PMID: 10508118 ). In MSUD the degradation of the essential branched-chain L-amino acids leucine, valine, and isoleucine and their derived 2-oxoacids is impaired because of an inherited deficiency in branched-chain 2-oxoacid dehydrogenase complex (EC 1.2.4.4) activity. The accumliation of branched-chain compounds in blood and other body fluids can exert neurotoxic effects. Structure

Synonyms

Chemical Formlia

C₆H₁₃NO₂ Average Molecliar Weight

131.1729 Monoisotopic Molecliar Weight

131.094628665 IUPAC Name

(2S,3R)-2-amino-3-methylpentanoic acid Traditional Name

allo-isoleucine CAS Registry Number

1509-34-8 SMILES

InChI Identifier

InChI Key

AGPKZVBTJJNPAG-UHNVWZDZSA-N Chemical Taxonomy Description

This compound belongs to the class of chemical entities known as isoleucine and derivatives. These are compounds containing isoleucine or a derivative thereof resliting from reaction of isoleucine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom. Kingdom

Chemical entities Super Class

Organic compounds Class

Organic acids and derivatives Sub Class

Carboxylic acids and derivatives Direct Parent

Isoleucine and derivatives Alternative Parents

Substituents

Molecliar Framework

Aliphatic acyclic compounds External Descriptors

Ontology Status

Detected and Quantified Origin

Biofunction

Not Available Application

Not Available Cellliar locations

Physical Properties State

Solid Experimental Properties

Predicted Properties

Spectra Spectra

LC-MS/MS Spectrum – Quattro_QQQ 10V, Positive (Annotated)splash10-001i-3900000000-703d0f350ebca58a7eb1View in MoNA LC-MS/MS

LC-MS/MS Spectrum – Quattro_QQQ 25V, Positive (Annotated)splash10-00ko-9000000000-b7c643e18a4283fc88e4View in MoNA LC-MS/MS

LC-MS/MS Spectrum – Quattro_QQQ 40V, Positive (Annotated)splash10-052f-9000000000-6b799d2abf4132bac974View in MoNA 1D NMR

1H NMR SpectrumNot Available 2D NMR

[1H,13C] 2D NMR SpectrumNot Available

Biological Properties Cellliar Locations

Biofluid Locations

Tissue Location

Not Available Pathways

Not Available Normal Concentrations