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Merck
CN

04609

琥珀酰左旋肉碱 锂盐

analytical standard

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关于此项目

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


存储类别

11 - Combustible Solids

wgk

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable



历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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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



全球贸易项目编号

货号GTIN
100293-25G04061838662507
100293-10G04061838662491
04609-50MG04061833414743
04609-10MG04061833378946