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04609

Supelco

O-Succinyl-L-carnitine lithium salt

analytical standard

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About This Item

Empirical Formula (Hill Notation):
C11H19NO6 · xLi+
Molecular Weight:
261.27 (free acid basis)
UNSPSC Code:
12352200
NACRES:
NA.24

grade

analytical standard

Quality Level

assay

≥94.0% (HPLC)

optical activity

[α]/D -16.0±3.0°, c = 0.1 in H2O

shelf life

limited shelf life, expiry date on the label

application(s)

clinical testing

format

neat

storage temp.

2-8°C

Biochem/physiol Actions

O-Succinyl-L-carnitine (C4DC) is a secondary metabolite that is not directly involved in the normal growth, development or reproduction of an organism.arises from. O-Succinyl-L-carnitine arises from the TCA cycle intermediate succinyl-CoA in plasma and liver. It is an O-acylcarnitine having succinyl as the acyl substituent. O-Succinyl-L-carnitine is used as biomarker for inborn metabolic deseases related to succinyl-CoA synthase function.

Storage Class

11 - Combustible Solids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable


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Succinyl-CoA:acetoacetate transferase deficiency: identification of a new patient with a neonatal onset and review of the literature.
Niezen-Koning, K.E., et al.
The Journal of Pediatrics, 156, 870-873 (1997)
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

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