50405
2-Methylbutyryl-L-carnitine
≥97.0% (HPLC)
別名:
(2R)-3-Carboxy-N,N,N-trimethyl-2-(2-methyl-1-oxobutoxy)-1-propanaminium inner salt
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この商品について
実験式（ヒル表記法）:
C12H23NO4
CAS番号:
分子量:
245.32
NACRES:
NA.26
PubChem Substance ID:
UNSPSC Code:
41116107
MDL number:
Beilstein/REAXYS Number:
5946881
製品名
2-Methylbutyryl-L-carnitine, ≥97.0% (HPLC)
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
SMILES string
C[N+](C)(C)C[C@H](OC(C(CC)C)=O)CC([O-])=O
InChI key
IHCPDBBYTYJYIL-QVDQXJPCSA-N
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
Quality Level
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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.
保管分類
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
適用法令
試験研究用途を考慮した関連法令を主に挙げております。化学物質以外については、一部の情報のみ提供しています。 製品を安全かつ合法的に使用することは、使用者の義務です。最新情報により修正される場合があります。WEBの反映には時間を要することがあるため、適宜SDSをご参照ください。
50405-VAR: + 50405-10MG: + 50405-50MG: + 50405-BULK:
jan
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
S Pontremoli et al.
Biochemical and biophysical research communications, 148(3), 1189-1195 (1987-11-13)
Isovalerylcarnitine (IVC) a product of the catabolism of L-leucine, is a potent activator of the Ca2+-dependent proteinase (calpain) of human neutrophils. At concentrations of Ca2+ in the low micromolar range, activation was 12 to 15-fold, and the activity exceeded that
M Fontaine et al.
Clinica chimica acta; international journal of clinical chemistry, 252(2), 109-122 (1996-08-30)
Carnitine levels and acylcarnitine profiles in a patient with mild multiple acyl-CoA dehydrogenase deficient beta-oxidation were compared with control results. Whereas blood and urine total carnitine levels were moderately decreased, blood esterified carnitine levels in the patient were about 2-fold
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
Prenatal diagnosis of isovaleric acidemia by fast atom bombardment and tandem mass spectrometry.
Y Shigematsu et al.
Clinica chimica acta; international journal of clinical chemistry, 203(2-3), 369-374 (1991-12-16)
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