ChemicalBook--->CAS DataBase List--->99159-87-2

99159-87-2

99159-87-2 Structure

99159-87-2 Structure
IdentificationBack 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 PropertiesBack Directory
[storage temp. ]

Hygroscopic, -20°C Freezer, Under inert atmosphere
[solubility ]

Methanol (Slightly), Water (Slightly)
[form ]

Solid
[pka]

3.391±0.10(predicted)
[color ]

White to Pale Yellow
[Optical Rotation]

[α]/D -17.0±2.0°, c =0.1 in H2O
[Stability:]

Very Hygroscopic
[Major Application]

clinical testing
[InChI]

1S/C12H23NO5/c1-9(2)6-11(16)18-12(17,7-10(14)15)8-13(3,4)5/h9,17H,6-8H2,1-5H3
[InChIKey]

DHRNMNHPSWBJEN-UHFFFAOYSA-N
[SMILES]

C[N+](C)(C)C[C@H](OC(CC(C)(O)C)=O)CC([O-])=O
Safety DataBack Directory
[WGK Germany ]

WGK 3
[HS Code ]

2921199090
[Storage Class]

11 - Combustible Solids
Hazard InformationBack 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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