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3-Hydroxyisovalerylcarnitine

CAT: 0931-TN7605-01Size: 10 mgDry Ice: NoHazardous: No
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CAT#:0931-TN7605-01Size:10 mg
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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CAS Number
99159-87-2
Target
Endogenous Metabolite
Related Pathways
Metabolism
Bioactivity
3-Hydroxyisovalerylcarnitine is a carnitine derivative that may serve as a novel biomarker for biotin deficiency in humans, with its concentration increasing in such conditions. Biotin deficiency reduces the activity of biotin-dependent 3-methylcrotonyl-CoA carboxylase, hindering the conversion of 3-methylcrotonyl-CoA into 3-methylglutaconyl-CoA, and impairing the leucine catabolism pathway, resulting in elevated plasma 3-Hydroxyisovalerylcarnitine concentrations [1].
Smiles
O([C@@H](C[N+](C)(C)C)CC([O-])=O)C(CC(C)(C)O)=O
Molecular Formula
C12H23NO5
Molecular Weight
261.32
Shipping Conditions
Ice Packs
Storage Temperature
-20°C

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