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MilliporeSigma

3-Hydroxyisovaleryl-L-carnitine analytical standard

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Synonyms: (2R)-3-Carboxy-2-(3-hydroxy-3-methyl-1-oxobutoxy)-N,N,N-trimethyl-1-propanaminium inner salt

Molecular Formula: C12H23NO5

Molecular Weight: 261.31

Linear Structural Formula: C12H23NO5

Purity: >=90.0% (HPLC)

Storage: 2-8C

Biochem Physiol Actions: 3-Hydroxyisovaleryl-L-carnitine is an endogenous metabolite that can result from impairment in the leucine catabolic pathway. 3-Hydroxyisovaleryl-L-carnitine was observed in plasma and urine of patient with isolated 3-methylcrotonyl-CoA carboxylase deficiency and is used as biomarker of biotin deficiency in humans and other metabolic disorders.

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Product Detail
Thomas No.
CHM02L298
Mfr. No.
91298-10MG
Description
91298-10MG, 3-Hydroxyisovaleryl-L-carnitine analytical standard
CAS Number
99159-87-2
list price/quantitytotal
$0.00
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