Grade
analytical standard
분석
≥96.5% (HPLC)
유통기한
limited shelf life, expiry date on the label
응용 분야
clinical testing
형식
neat
SMILES string
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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일반 설명
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.
애플리케이션
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.
Storage Class Code
11 - Combustible Solids
WGK
WGK 3
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
가장 최신 버전 중 하나를 선택하세요:
Diagnosis and management of glutaric aciduria type I
Journal of inherited metabolic disease, 21(4), 326-340 (1998)
Two uneventful pregnancies in a woman with Glutaric aciduria type 1
JIMD Reports, 41(4), 29-36 (2018)
Induction of the mitochondrial permeability transition in vitro by short-chain carboxylic acids
Biochemical and Biophysical Research Communications, 272, 431-435 (2018)
Prevention of cerebral palsy in glutaric aciduria type 1 by dietary management
Archives of Disease in Childhood, 82(1), 67-70 (2000)
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