All Photos(1)

102687

Sigma-Aldrich

Ethylmalonic acid

97%

Synonym(s):
α-Carboxybutyric acid, 2-Ethylpropanedioic acid
Linear Formula:
C2H5CH(COOH)2
CAS Number:
Molecular Weight:
132.11
Beilstein:
774334
EC Number:
MDL number:
PubChem Substance ID:
NACRES:
NA.23

Quality Level

assay

97%

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

Signal Word

Warning

Hazard Statements

Hazard Classifications

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

Target Organs

Respiratory system

Storage Class Code

11 - Combustible Solids

WGK Germany

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US),Eyeshields,Gloves

Certificate of Analysis

Certificate of Origin

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...
B Merinero et al.
Journal of inherited metabolic disease, 29(5), 685-685 (2006-08-15)
High concentrations of butyryl/isobutyrylcarnitine (C(4)-carnitine) in plasma with increase of ethylmalonic acid (EMA) in urine point to different genetic entities, and further investigations are required to differentiate the possible underlying defect. Here we report three unrelated cases, two neurologically affected...
Lessons to learn from rare inborn errors of metabolism.
B Plecko
Neuropediatrics, 38(2), 59-60 (2007-08-23)

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