2-Methylbutyroylcarnitine
IUPAC 名称
3-[(2-methylbutanoyl)oxy]-4-(trimethylazaniumyl)butanoate
描述
Usually not detected in normal individuals, the elevation of 2-methylbutyrylcarnitine suggests a deficiency of a dehydrogenase specific for isobutyryl-CoA, important in the differential diagnosis of Branched Chain Organic Acidurias by Analysis of Urinary Organic Acids and Acylcarnitines in Plasma or Dried Blood Spots (The Metabolic and Molecular Bases of Inherited Disease, Chapter 93) [HMDB]
异构
CCC(C)C(=O)OC(CC([O-])=O)C[N+](C)(C)C
InChI标识符
InChI=1S/C12H23NO4/c1-6-9(2)12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3
键
IHCPDBBYTYJYIL-UHFFFAOYSA-N