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Merck
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95187

Supelco

Methylmalonyl-L-carnitine lithium salt

analytical standard

Sinónimos:

(2R)-3-Carboxy-2-(2-carboxy-1-oxopropoxy)-N,N,N-trimethyl-1-propanaminium inner salt lithium salt, O-Methylmalonyl-L-carnitine lithium salt

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About This Item

Fórmula empírica (notación de Hill):
C11H19NO6 · xLi+
Número de CAS:
251976-17-7
Peso molecular:
261.27 (free acid basis)
Código UNSPSC:
41116107

grado

analytical standard

Nivel de calidad

100

Análisis

≥90.0% (HPLC)

formulario

solid

actividad óptica

[α]/D -33.0±2.0°, c = 0.1 in H2O

caducidad

limited shelf life, expiry date on the label

aplicaciones

clinical testing

temp. de almacenamiento

2-8°C

cadena SMILES

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

Acciones bioquímicas o fisiológicas

O-Methylmalonyl-L-carnitine belongs to the class of acyl carnitines. Acyl carnitines contain a fatty acid with the carboxylic acid attached to carnitine through an ester bond. The level of methylmalonyl carnitine has been found to be increased in patients with methylmalonuric aciduria (MMA), one of the most frequent organic acidurias caused by enzymatic defects involved in the catabolism of branched-chain amino acids.

Código de clase de almacenamiento

11 - Combustible Solids

Clase de riesgo para el agua (WGK)

WGK 3

Punto de inflamabilidad (°F)

Not applicable

Punto de inflamabilidad (°C)

Not applicable

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Certificados de análisis (COA)

Lot/Batch Number
BCCC8148
BCBS2060

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Cristiano Rizzo et al.
Clinica chimica acta; international journal of clinical chemistry, 429, 30-33 (2013-11-26)
Methylmalonic aciduria (MMA) is one of the most frequent organic acidurias, a class of diseases caused by enzymatic defects mainly involved in the catabolism of branched-chain amino acids. Recently, mild MMA and C4-dicarboxylyl-carnitine (C4DC-C) accumulation have been reported in patients
William R Wikoff et al.
Clinical chemistry, 53(12), 2169-2176 (2007-10-24)
We applied untargeted mass spectrometry-based metabolomics to the diseases methylmalonic acidemia (MMA) and propionic acidemia (PA). We used a screening platform that used untargeted, mass-based metabolomics of methanol-extracted plasma to find significantly different molecular features in human plasma samples from

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