102687
Ethylmalonic acid
97%
Synonym(s):
α-Carboxybutyric acid, 2-Ethylpropanedioic acid
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5 G
$70.50
50 G
$361.00
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5 G
$70.50
50 G
$361.00
About This Item
Linear Formula:
C2H5CH(COOH)2
CAS Number:
Molecular Weight:
132.11
Beilstein/REAXYS Number:
774334
EC Number:
MDL number:
UNSPSC Code:
12162002
PubChem Substance ID:
NACRES:
NA.23
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Quality Level
assay
97%
form
solid
mp
112-114 °C (lit.)
SMILES string
CCC(C(O)=O)C(O)=O
InChI
1S/C5H8O4/c1-2-3(4(6)7)5(8)9/h3H,2H2,1H3,(H,6,7)(H,8,9)
InChI key
UKFXDFUAPNAMPJ-UHFFFAOYSA-N
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signalword
Warning
hcodes
Hazard Classifications
Eye Irrit. 2 - Skin Irrit. 2 - STOT SE 3
target_organs
Respiratory system
Storage Class
11 - Combustible Solids
wgk_germany
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
dust mask type N95 (US), Eyeshields, Gloves
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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
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
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
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
Lada Cindro Heberle et al.
Brain & development, 28(5), 329-331 (2005-12-27)
Ethylmalonic encephalopathy is a rare metabolic disease presenting in infancy with developmental delay, acrocyanosis, petechiae, chronic diarrhea and early death. The biochemical characteristics of this autosomal recessive disease are urinary organic acid abnormalities. Recently it has been found to be
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