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102687

Sigma-Aldrich

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:
601-75-2
Molecular Weight:
132.11
Beilstein:
774334
EC Number:
210-007-2
MDL number:
MFCD00002668
UNSPSC Code:
12162002
PubChem Substance ID:
24846585
NACRES:
NA.23

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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Related Categories

Pictograms

Exclamation mark
GHS07

Signal Word

Warning

Hazard Statements

H315,H319,H335

Hazard Classifications

Eye Irrit. 2 - Skin Irrit. 2 - STOT SE 3

Target Organs

Respiratory system

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

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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)
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
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
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
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
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