91298
3-Hydroxyisovaleryl-L-carnitine
analytical standard
Synonym(s):
(2R)-3-Carboxy-2-(3-hydroxy-3-methyl-1-oxobutoxy)-N,N,N-trimethyl-1-propanaminium inner salt
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About This Item
Empirical Formula (Hill Notation):
C12H23NO5
CAS Number:
Molecular Weight:
261.31
NACRES:
NA.24
UNSPSC Code:
41116107
MDL number:
Beilstein/REAXYS Number:
10549761
grade
analytical standard
Quality Segment
assay
≥90.0% (HPLC)
optical activity
[α]/D -17.0±2.0°, c = 0.1 in H2O
shelf life
limited shelf life, expiry date on the label
impurities
≤15% water (calc. on elemental analysis)
application(s)
clinical testing
format
neat
storage temp.
2-8°C
SMILES string
C[N+](C)(C)C[C@H](OC(CC(C)(O)C)=O)CC([O-])=O
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
InChI key
DHRNMNHPSWBJEN-UHFFFAOYSA-N
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.
Other Notes
Find a digital Reference Material for this product available on our online platform ChemisTwin® for NMR. You can use this digital equivalent on ChemisTwin® for your sample identity confirmation and compound quantification (with digital external standard). An NMR spectrum of this substance can be viewed and an online comparison against your sample can be performed with a few mouseclicks. Learn more here and start your free trial.
Storage Class
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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