| Identification | Back Directory | [Name]
Hydroxyisovaleroyl Carnitine | [CAS]
99159-87-2 | [Synonyms]
IGLHHSKNBDXCEY-SECBINFHSA-N
Hydroxyisovaleroyl Carnitine
(2R)-3-Hydroxyisovaleroyl Carnitine | [Molecular Formula]
C12H23NO5 | [MOL File]
99159-87-2.mol | [Molecular Weight]
261.32 |
| Chemical Properties | Back Directory | [storage temp. ]
Hygroscopic, -20°C Freezer, Under inert atmosphere | [solubility ]
Methanol (Slightly), Water (Slightly) | [form ]
Solid | [color ]
White to Pale Yellow | [Optical Rotation]
[α]/D -17.0±2.0°, c =0.1 in H2O | [Stability:]
Very Hygroscopic |
| Hazard Information | Back Directory | [Uses]
Hydroxyisovaleroyl Carnitine is used as a diagnostic tool to identify the presence of orofacial clefts. Also used in the screening and diagnostics of inborn errors of metabolism. | [Definition]
ChEBI: Hydroxyisovaleroyl carnitine is an O-acylcarnitine. | [Biological Activity]
3-Hydroxyisovaleryl-L-carnitine is an endogenous metabolite th at 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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| Company Name: |
Merck KGaA
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| Tel: |
21-20338288 |
| Website: |
www.sigmaaldrich.cn |
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