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Details

Stereochemistry ACHIRAL
Molecular Formula C5H8O4
Molecular Weight 132.1146
Optical Activity NONE
Defined Stereocenters 0 / 0
E/Z Centers 0
Charge 0

SHOW SMILES / InChI
Structure of ETHYLMALONIC ACID

SMILES

CCC(C(O)=O)C(O)=O

InChI

InChIKey=UKFXDFUAPNAMPJ-UHFFFAOYSA-N
InChI=1S/C5H8O4/c1-2-3(4(6)7)5(8)9/h3H,2H2,1H3,(H,6,7)(H,8,9)

HIDE SMILES / InChI

Molecular Formula C5H8O4
Molecular Weight 132.1146
Charge 0
Count
Stereochemistry ACHIRAL
Additional Stereochemistry No
Defined Stereocenters 0 / 0
E/Z Centers 0
Optical Activity NONE

Approval Year

PubMed

PubMed

TitleDatePubMed
Risk factors of ocular involvement in children with mitochondrial respiratory chain complex defect.
2010-12
Ethylene glycol monomethyl ether-induced toxicity is mediated through the inhibition of flavoprotein dehydrogenase enzyme family.
2010-12
A systems biology approach uncovers cellular strategies used by Methylobacterium extorquens AM1 during the switch from multi- to single-carbon growth.
2010-11-24
6-[Bis(ethoxycarbonyl)methyl]-6-deoxy-1,2;3,4-di-O-isopropyl-idene-d-galacto-pyran-ose.
2010-11-17
Pathogenesis and treatment of mitochondrial myopathies: recent advances.
2010-10
Clinical aspects of short-chain acyl-CoA dehydrogenase deficiency.
2010-10
Combined treatment with oral metronidazole and N-acetylcysteine is effective in ethylmalonic encephalopathy.
2010-08
Antioxidant dysfunction: potential risk for neurotoxicity in ethylmalonic aciduria.
2010-06
Silent increase of urinary ethylmalonic acid is an indicator of nonspecific brain dysfunction.
2010-05
Flavin adenine dinucleotide status and the effects of high-dose riboflavin treatment in short-chain acyl-CoA dehydrogenase deficiency.
2010-03
Promotion of lipid and protein oxidative damage in rat brain by ethylmalonic acid.
2010-02
Fasting and fat-loading tests provide pathophysiological insight into short-chain acyl-coenzyme a dehydrogenase deficiency.
2010-01
Inborn errors of energy metabolism associated with myopathies.
2010
Chronic early postnatal administration of ethylmalonic acid to rats causes behavioral deficit.
2009-02-11
Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy.
2009-02
Short-chain acyl-coenzyme A dehydrogenase deficiency.
2008-12
The ACADS gene variation spectrum in 114 patients with short-chain acyl-CoA dehydrogenase (SCAD) deficiency is dominated by missense variations leading to protein misfolding at the cellular level.
2008-08
[Short-chain acyl-CoA dehydrogenase deficiency (SCADD): relatively high prevalence in the Netherlands and strongly variable fenotype; neonatal screening not indicated].
2008-07-26
2-(4-Ethoxy-benzyl-idene)butanoic acid.
2008-07-12
Severe infantile hypotonia with ethylmalonic aciduria: case report.
2008-06
Acquired multiple Acyl-CoA dehydrogenase deficiency in 10 horses with atypical myopathy.
2008-05
(E)-2-(2-Fluoro-benzyl-idene)butanoic acid.
2008-03-20
A new case of short-chain acyl-CoA dehydrogenase deficiency: clinical, biochemical, genetic and (1)H-NMR spectroscopic studies.
2007-12
Equine biochemical multiple acyl-CoA dehydrogenase deficiency (MADD) as a cause of rhabdomyolysis.
2007-08
Ethylmalonic encephalopathy: clinical and biochemical observations.
2007-04
Lessons to learn from rare inborn errors of metabolism.
2007-04
Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency.
2007-02
Taxonomic revision of Aspergillus section Clavati based on molecular, morphological and physiological data.
2007
Single-nucleotide variations in the genes encoding the mitochondrial Hsp60/Hsp10 chaperone system and their disease-causing potential.
2007
A case of ethylmalonic encephalopathy with atypical clinical and biochemical presentation.
2006-12
Persistent increase of plasma butyryl/isobutyrylcarnitine concentrations as marker of SCAD defect and ethylmalonic encephalopathy.
2006-10
Clinical, biochemical, and genetic heterogeneity in short-chain acyl-coenzyme A dehydrogenase deficiency.
2006-08-23
Ethylmalonic encephalopathy-report of two cases.
2006-06
ETHE1 mutations are specific to ethylmalonic encephalopathy.
2006-04
Effect of in vivo administration of ethylmalonic acid on energy metabolism in rat tissues.
2006-03
Inhibition of the electron transport chain and creatine kinase activity by ethylmalonic acid in human skeletal muscle.
2006-03
Metabolomic and bioinformatic analyses in asphyxiated neonates.
2006-03
Perioperative management of a child with short-chain acyl-CoA dehydrogenase deficiency.
2005-09
Acyl-CoA dehydrogenase deficiency: varieties with neurological involvement.
2005-03
Clinical, biochemical, magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H MRS) findings in a fourth case of combined D- and L-2 hydroxyglutaric aciduria.
2005
The putative elaiophylin biosynthetic gene cluster in Streptomyces sp. DSM4137 is adjacent to genes encoding adenosylcobalamin-dependent methylmalonyl CoA mutase and to genes for synthesis of cobalamin.
2004-09-30
Recent developments and new applications of tandem mass spectrometry in newborn screening.
2004-08
The role of methionine in ethylmalonic encephalopathy with petechiae.
2004-04
Ethylmalonic encephalopathy is caused by mutations in ETHE1, a gene encoding a mitochondrial matrix protein.
2004-02
[Late-onset riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II)].
2003-12
Short-chain Acyl-CoA dehydrogenase deficiency: studies in a large family adding to the complexity of the disorder.
2003-11
A comparison of in vitro acylcarnitine profiling methods for the diagnosis of classical and variant short chain acyl-CoA dehydrogenase deficiency.
2003-11
Mutation analysis of copper transporter genes in patients with ethylmalonic encephalopathy, mitochondriopathies and copper deficiency phenotypes.
2003
Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency.
1995-06
Age-related reference values for urinary organic acids in a healthy Turkish pediatric population.
1994-06
Patents
Substance Class Chemical
Created
by admin
on Mon Mar 31 19:54:53 GMT 2025
Edited
by admin
on Mon Mar 31 19:54:53 GMT 2025
Record UNII
432NF49DFG
Record Status Validated (UNII)
Record Version
  • Download
Name Type Language
ETHYLMALONIC ACID
Systematic Name English
NSC-96615
Preferred Name English
Code System Code Type Description
ECHA (EC/EINECS)
210-007-2
Created by admin on Mon Mar 31 19:54:53 GMT 2025 , Edited by admin on Mon Mar 31 19:54:53 GMT 2025
PRIMARY
FDA UNII
432NF49DFG
Created by admin on Mon Mar 31 19:54:53 GMT 2025 , Edited by admin on Mon Mar 31 19:54:53 GMT 2025
PRIMARY
EPA CompTox
DTXSID00208842
Created by admin on Mon Mar 31 19:54:53 GMT 2025 , Edited by admin on Mon Mar 31 19:54:53 GMT 2025
PRIMARY
CAS
601-75-2
Created by admin on Mon Mar 31 19:54:53 GMT 2025 , Edited by admin on Mon Mar 31 19:54:53 GMT 2025
PRIMARY
NSC
96615
Created by admin on Mon Mar 31 19:54:53 GMT 2025 , Edited by admin on Mon Mar 31 19:54:53 GMT 2025
PRIMARY
MESH
C038080
Created by admin on Mon Mar 31 19:54:53 GMT 2025 , Edited by admin on Mon Mar 31 19:54:53 GMT 2025
PRIMARY
PUBCHEM
11756
Created by admin on Mon Mar 31 19:54:53 GMT 2025 , Edited by admin on Mon Mar 31 19:54:53 GMT 2025
PRIMARY