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Merck

53439

Supelco

葡萄糖酸

analytical standard

别名:

2-戊烯二酸

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About This Item

线性分子式:
HOOCCH2CH=CHCOOH
CAS号:
分子量:
130.10
Beilstein:
1759560
MDL编号:
UNSPSC代码:
41116107

等级

analytical standard

方案

≥96.5% (HPLC)

保质期

limited shelf life, expiry date on the label

应用

clinical testing

包装形式

neat

SMILES字符串

OC(=O)C\C=C\C(O)=O

InChI

1S/C5H6O4/c6-4(7)2-1-3-5(8)9/h1-2H,3H2,(H,6,7)(H,8,9)/b2-1+

InChI key

XVOUMQNXTGKGMA-OWOJBTEDSA-N

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一般描述

Glutaconic acid is a short chain carboxylic acid which accumulates in the physiological body fluids in patients with deficiency of glutaryl-CoA dehydrogenase leading to glutaric aciduria type 1 disorder (GA1). It finds applications as a diagnostic biomarker since it is found in increased concentrations in the urine of patients with GA1. It can be detected by gas chromatography-mass spectrometry in biological fluids including urine, cerebrospinal fluid, and serum.

应用

Glutaconic acid may be used as a standard to investigate its role in the induction of the mitochondrial permeability transition (MPT) in the isolated rat liver mitochondria in vitro.

储存分类代码

11 - Combustible Solids

WGK

WGK 3

闪点(°F)

Not applicable

闪点(°C)

Not applicable


历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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访问文档库

Diagnosis and management of glutaric aciduria type I
Baric I, et al.
Journal of inherited metabolic disease, 21(4), 326-340 (1998)
Two uneventful pregnancies in a woman with Glutaric aciduria type 1
Stepien MK, et al.
JIMD Reports, 41(4), 29-36 (2018)
Prevention of cerebral palsy in glutaric aciduria type 1 by dietary management
Monavari AA and Naugten RE
Archives of Disease in Childhood, 82(1), 67-70 (2000)
Induction of the mitochondrial permeability transition in vitro by short-chain carboxylic acids
Palmeira MC, et al.
Biochemical and Biophysical Research Communications, 272, 431-435 (2018)

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