等級
analytical standard
化驗
≥96.5% (HPLC)
儲存期限
limited shelf life, expiry date on the label
應用
clinical testing
格式
neat
SMILES 字串
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 密鑰
XVOUMQNXTGKGMA-OWOJBTEDSA-N
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一般說明
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.
應用
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.
儲存類別代碼
11 - Combustible Solids
水污染物質分類（WGK）
WGK 3
閃點（°F）
Not applicable
閃點（°C）
Not applicable
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)
Diagnosis and management of glutaric aciduria type I
Baric I, et al.
Journal of inherited metabolic disease, 21(4), 326-340 (1998)
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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