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95187

Supelco

Methylmalonyl-L-carnitine lithium salt

analytical standard

Synonym(s):

(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

Empirical Formula (Hill Notation):
C11H19NO6 · xLi+
CAS Number:
251976-17-7
Molecular Weight:
261.27 (free acid basis)
UNSPSC Code:
41116107
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grade

analytical standard

Quality Level

100

Assay

≥90.0% (HPLC)

form

solid

optical activity

[α]/D -33.0±2.0°, c = 0.1 in H2O

shelf life

limited shelf life, expiry date on the label

application(s)

clinical testing

storage temp.

2-8°C

SMILES string

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

Biochem/physiol Actions

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.

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
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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