102687
Ethylmalonic acid
97%
Synonym(s):
α-Carboxybutyric acid, 2-Ethylpropanedioic acid
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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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Maja Di Rocco et al.
Molecular genetics and metabolism, 89(4), 395-397 (2006-07-11)
A child is reported presenting with a clinical picture suggestive of genetic connective tissue disorders (vascular fragility, articular hyperlaxity, delayed motor development, and normal cognitive development), an absence of pathological ethylmalonic acid excretion during inter-critical phases and a homozygous R163W
Patrícia Fernanda Schuck et al.
Neurochemical research, 35(2), 298-305 (2009-09-17)
High concentrations of ethylmalonic acid are found in tissues and biological fluids of patients affected by ethylmalonic encephalopathy, deficiency of short-chain acyl-CoA dehydrogenase activity and other illnesses characterized by developmental delay and neuromuscular symptoms. The pathophysiological mechanisms responsible for the
Carlo Viscomi et al.
Nature medicine, 16(8), 869-871 (2010-07-27)
Ethylmalonic encephalopathy is caused by mutations in ETHE1, a mitochondrial matrix sulfur dioxygenase, leading to failure to detoxify sulfide, a product of intestinal anaerobes and, in trace amounts, tissues. Metronidazole, a bactericide, or N-acetylcysteine, a precursor of sulfide-buffering glutathione, substantially
Patrícia Fernanda Schuck et al.
Synapse (New York, N.Y.), 67(3), 111-117 (2012-11-20)
Ethylmalonic acid (EMA) accumulates in tissues of patients affected by short-chain acyl-CoA dehydrogenase deficiency and ethylmalonic encephalopathy, illnesses characterized by variable neurological symptoms. In this work, we investigated the in vitro and in vivo EMA effects on Na(+), K(+)-ATPase (NAK)
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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