102687
乙基丙二酸
97%
别名:
α-Carboxybutyric acid, 2-Ethylpropanedioic acid
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线性分子式:
C2H5CH(COOH)2
化学文摘社编号:
分子量:
132.11
UNSPSC Code:
12162002
NACRES:
NA.23
PubChem Substance ID:
EC Number:
210-007-2
Beilstein/REAXYS Number:
774334
MDL number:
产品名称
乙基丙二酸, 97%
InChI key
UKFXDFUAPNAMPJ-UHFFFAOYSA-N
InChI
1S/C5H8O4/c1-2-3(4(6)7)5(8)9/h3H,2H2,1H3,(H,6,7)(H,8,9)
SMILES string
CCC(C(O)=O)C(O)=O
assay
97%
form
solid
mp
112-114 °C (lit.)
Quality Level
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signalword
Warning
hcodes
Hazard Classifications
Eye Irrit. 2 - Skin Irrit. 2 - STOT SE 3
target_organs
Respiratory system
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
dust mask type N95 (US), Eyeshields, Gloves
Patrícia Fernanda Schuck et al.
Behavioural brain research, 197(2), 364-370 (2008-10-28)
High concentrations of ethylmalonic acid (EMA) are found in tissues and biological fluids of patients affected by ethylmalonic encephalopathy (EE), as well as by deficiency of short-chain acyl-CoA dehydrogenase (SCAD) activity and other illnesses characterized by developmental delay and other
Christina B Pedersen et al.
Journal of inherited metabolic disease, 33(3), 211-222 (2010-05-06)
Mitochondrial dysfunction and oxidative stress are central to the molecular basis of several human diseases associated with neuromuscular disabilities. We hypothesize that mitochondrial dysfunction also contributes to the neuromuscular symptoms observed in patients with ethylmalonic aciduria and homozygosity for ACADS
Imad Dweikat et al.
Metabolic brain disease, 27(4), 613-616 (2012-05-16)
Ethylmalonic encephalopathy (EE) is a rare autosomal recessive disorder caused by mutations in the ETHE1 gene and characterized by chronic diarrhea, encephalopathy, relapsing petechiae and acrocyanosis. Nephrotic syndrome has been described in an infant with EE but the renal histology
Bianca T van Maldegem et al.
Pediatric research, 67(3), 304-308 (2009-12-03)
Short-chain acyl-CoA dehydrogenase deficiency (SCADD) is an inborn error, biochemically characterized by increased plasma butyrylcarnitine (C4-C) concentration and increased ethylmalonic acid (EMA) excretion and caused by rare mutations and/or common gene variants in the SCAD encoding gene. Although its clinical
Raffaella Zannolli et al.
NMR in biomedicine, 23(4), 353-358 (2010-02-27)
Our aim was to compare urinary ethylmalonic acid (EMA) levels in subjects who had no apparent clinical reason to have increased levels of this substance but were suffering from non-specific CNS impairment, and healthy controls. Urinary EMA concentrations detected by
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