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51085

Sigma-Aldrich

Isobutyryl-L-carnitine

≥97.0% (TLC)

Sinónimos:

(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

Fórmula empírica (notación de Hill):
C11H21NO4
Número de CAS:
25518-49-4
Peso molecular:
231.29
Beilstein/REAXYS Number:
4138517
MDL number:
MFCD01674829
UNSPSC Code:
41116107
PubChem Substance ID:
329757668
NACRES:
NA.26

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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Categorías relacionadas

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

11 - Combustible Solids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

Certificados de análisis (COA)

Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»

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Sigma-Aldrich

441554

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L-Tryptophan reagent grade, ≥98% (HPLC)

Sigma-Aldrich

T0254

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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
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
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
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
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