50405
2-Methylbutyryl-L-carnitine
≥97.0% (HPLC)
Synonym(s):
(2R)-3-Carboxy-N,N,N-trimethyl-2-(2-methyl-1-oxobutoxy)-1-propanaminium inner salt
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About This Item
Empirical Formula (Hill Notation):
C12H23NO4
CAS Number:
Molecular Weight:
245.32
NACRES:
NA.26
PubChem Substance ID:
UNSPSC Code:
41116107
MDL number:
Beilstein/REAXYS Number:
5946881
Product Name
2-Methylbutyryl-L-carnitine, ≥97.0% (HPLC)
Quality Level
assay
≥97.0% (HPLC)
form
powder or crystals
optical activity
[α]/D -20±2°, c = 0.1 in H2O
impurities
≤10% water
color
white to off-white
storage temp.
2-8°C
SMILES string
C[N+](C)(C)C[C@H](OC(C(CC)C)=O)CC([O-])=O
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/t9?,10-/m1/s1
InChI key
IHCPDBBYTYJYIL-QVDQXJPCSA-N
Biochem/physiol Actions
2-Methylbutyryl-L-carnitine is not usually 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.
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Storage Class
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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Dietrich Matern et al.
Pediatrics, 112(1 Pt 1), 74-78 (2003-07-03)
2-methylbutyryl-CoA dehydrogenase deficiency, also known as short/branched-chain acyl-CoA dehydrogenase (SBCAD) deficiency, is a recently described autosomal recessive disorder of L-isoleucine metabolism. Only 4 affected individuals in 2 families have been described. One patient developed athetoid cerebral palsy, and another had
R A Chalmers et al.
Pediatric research, 18(12), 1325-1328 (1984-12-01)
Concentrations of l-carnitine and acylcarnitines have been determined in urine from patients with disorders of organic acid metabolism associated with an intramitochondrial accumulation of acyl-CoA intermediates. These included propionic acidemia, methylmalonic aciduria, isovaleric acidemia, multicarboxylase deficiency, 3-hydroxy-3-methylglutaric aciduria, methylacetoacetyl-CoA thiolase
K Kidouchi et al.
Clinica chimica acta; international journal of clinical chemistry, 173(3), 263-272 (1988-04-29)
A quantitative analysis for urinary acylcarnitines in a patient with neonatal multiple acyl-CoA dehydrogenation deficiency is described. This method (liquid chromatography) can quantify twelve acylcarnitines including glutarylcarnitine and 3 isomeric acylcarnitines (butyryl-1, valeryl- and octanoylisomer) in urine. Before and up