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Merck
CN

51085

Isobutyryl-L-carnitine

≥97.0% (TLC)

Synonym(s):

(2R)-3-Carboxy-N,N,N-trimethyl-2-(2-methyl-1-oxopropoxy)-1-propanaminium inner salt, L-Carnitine isobutyryl ester, iC4-Carnitine

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About This Item

Empirical Formula (Hill Notation):
C11H21NO4
CAS Number:
25518-49-4
Molecular Weight:
231.29
Beilstein:
4138517
MDL number:
MFCD01674829
UNSPSC Code:
41116107
PubChem Substance ID:
329757668
NACRES:
NA.26

Key Documents

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Product Name

Isobutyryl-L-carnitine, ≥97.0% (HPLC)

Quality Level

100

Assay

≥97.0% (HPLC)

form

powder

optical activity

[α]/D -22±2°, c = 1 in H2O

impurities

≤10% water

color

white

storage temp.

2-8°C

SMILES string

C[N+](C)(C)C[C@H](OC(C(C)C)=O)CC([O-])=O

InChI

1S/C11H21NO4/c1-8(2)11(15)16-9(6-10(13)14)7-12(3,4)5/h8-9H,6-7H2,1-5H3/t9-/m1/s1

InChI key

LRCNOZRCYBNMEP-SECBINFHSA-N

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Biochem/physiol Actions

Isobutyryl-L-carnitine is a product of the acyl-CoA dehydrogenases (ACADs), which are a group of mitochondrial enzymes involved in the metabolism of fatty acids or branched-chain amino acids.

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

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Certificates of Analysis (COA)

Lot/Batch Number
BCCL7797
BCCH3319
BCBP2550V
BCBP2550

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K Matsumoto et al.
Clinica chimica acta; international journal of clinical chemistry, 216(1-2), 135-143 (1993-07-16)
An immobilized enzyme reactor, made up acylcarnitine hydrolase, carnitine dehydrogenase and diaphorase in sequence, was developed for the sensitive and selective determination of urinary free and individual acylcarnitines by a reversed-phase high-performance liquid chromatography. A 100-microliter urine sample was directly
Dwight D Koeberl et al.
Pediatric research, 54(2), 219-223 (2003-05-09)
Tandem mass spectrometry was adopted for newborn screening by North Carolina in April 1999. Since then, three infants with short-chain acyl-CoA dehydrogenase (SCAD) and one with isobutyryl-CoA dehydrogenase deficiency were detected on the basis of elevated butyrylcarnitine/isobutyrylcarnitine (C4-carnitine) concentrations in
M Fontaine et al.
Clinica chimica acta; international journal of clinical chemistry, 273(2), 161-170 (1998-07-10)
Here we report on a patient with severe ("non-classic") carnitine palmitoyltransferase type II (CPT II) deficiency. Hypoglycemia prompted by an infectious episode and associated with non-ketotic dicarboxylic aciduria orientated diagnosis towards beta-oxidation deficiency disorders. Blood carnitine levels revealed a secondary
N Sugiyama et al.
Acta paediatrica Japonica : Overseas edition, 32(4), 410-416 (1990-08-01)
A large quantity of propionylcarnitine in the urine of patients with propionic acidemia and methylmalonic aciduria was demonstrated. The amount excreted depended on the administered L-carnitine dose from 25 to 75 mg/kg/day. A high level of propionylcarnitine was also detected
L Vallée et al.
European journal of pediatrics, 153(8), 598-603 (1994-08-01)
We describe on a 3-year-old child referred for evaluation and therapy of a cerebral vascular accident with residual hemiplegia and partial epilepsy. Metabolic investigations initially showed normal urinary organic acids as well as normal blood and urinary amino acids. Blood
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