grade
analytical standard
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
Quality Level
相关类别
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
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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