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Merck
CN
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文件

04609

Supelco

琥珀酰左旋肉碱 锂盐

analytical standard

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About This Item

经验公式（希尔记法）:
C11H19NO6 · xLi+
分子量:
261.27 (free acid basis)
UNSPSC代码:
41116107
NACRES:
NA.24

等级

analytical standard

质量水平

100

检测方案

≥94.0% (HPLC)

旋光性

[α]/D -16.0±3.0°, c = 0.1 in H2O

保质期

limited shelf life, expiry date on the label

应用

clinical testing

格式

neat

储存温度

2-8°C

相关类别

生化/生理作用

O-琥珀酰-L-肉碱（C4DC）是次生代谢产物，不直接参与生物的正常生长、发育或繁殖。O-琥珀酰-L-肉碱来自血浆和肝脏中的 TCA 循环中间的琥珀酰-CoA。它是具有琥珀酰基作为酰基取代基的 O-酰基肉碱。O-琥珀酰-L-肉碱被用作与琥珀酰-CoA 合酶功能有关的先天性代谢疾病的生物标记。

WGK

WGK 3

闪点(°F)

Not applicable

闪点(°C)

Not applicable

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分析证书(COA)

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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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