ETHYLMALONIC ACID

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 Search

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

Title	Date	PubMed
Age-related reference values for urinary organic acids in a healthy Turkish pediatric population.	1994 Jun	8087979
Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency.	1995 Jun	7776094
Therapeutic trial in the first three Asian cases of ethylmalonic encephalopathy: response to riboflavin.	2001 Dec	11916321
Hypoglycaemia and elevated urine ethylmalonic acid in a child homozygous for the short-chain acyl-CoA dehydrogenase 625G > A gene variation.	2002	12061367
Inhibition of creatine kinase activity in vitro by ethylmalonic acid in cerebral cortex of young rats.	2002 Dec	12515316
Recurrent vomiting and ethylmalonic aciduria associated with rare mutations of the short-chain acyl-CoA dehydrogenase gene.	2003	12872838
Short-chain Acyl-CoA dehydrogenase deficiency: studies in a large family adding to the complexity of the disorder.	2003 Nov	14595061
A comparison of in vitro acylcarnitine profiling methods for the diagnosis of classical and variant short chain acyl-CoA dehydrogenase deficiency.	2003 Nov	14568186
Acyl-CoA dehydrogenase deficiency: varieties with neurological involvement.	2005 Mar	15739728
Perioperative management of a child with short-chain acyl-CoA dehydrogenase deficiency.	2005 Sep	16101709
ETHE1 mutations are specific to ethylmalonic encephalopathy.	2006 Apr	16183799
Effect of in vivo administration of ethylmalonic acid on energy metabolism in rat tissues.	2006 Mar	16773468
Taxonomic revision of Aspergillus section Clavati based on molecular, morphological and physiological data.	2007	18490946
Single-nucleotide variations in the genes encoding the mitochondrial Hsp60/Hsp10 chaperone system and their disease-causing potential.	2007	17072495
Ethylmalonic encephalopathy: clinical and biochemical observations.	2007 Apr	17712735
Lessons to learn from rare inborn errors of metabolism.	2007 Apr	17712731
A new case of short-chain acyl-CoA dehydrogenase deficiency: clinical, biochemical, genetic and (1)H-NMR spectroscopic studies.	2007 Dec	18175080
Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency.	2007 Feb	17304052
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 Aug	18523805
Short-chain acyl-coenzyme A dehydrogenase deficiency.	2008 Dec	18977676
2-(4-Ethoxy-benzyl-idene)butanoic acid.	2008 Jul 12	21203172
[Short-chain acyl-CoA dehydrogenase deficiency (SCADD): relatively high prevalence in the Netherlands and strongly variable fenotype; neonatal screening not indicated].	2008 Jul 26	18714522
Severe infantile hypotonia with ethylmalonic aciduria: case report.	2008 Jun	18539996
(E)-2-(2-Fluoro-benzyl-idene)butanoic acid.	2008 Mar 20	21202123
Acquired multiple Acyl-CoA dehydrogenase deficiency in 10 horses with atypical myopathy.	2008 May	18406615
Chronic early postnatal administration of ethylmalonic acid to rats causes behavioral deficit.	2009 Feb 11	18950661
Inborn errors of energy metabolism associated with myopathies.	2010	20589068
Combined treatment with oral metronidazole and N-acetylcysteine is effective in ethylmalonic encephalopathy.	2010 Aug	20657580
Risk factors of ocular involvement in children with mitochondrial respiratory chain complex defect.	2010 Dec	21253313
Ethylene glycol monomethyl ether-induced toxicity is mediated through the inhibition of flavoprotein dehydrogenase enzyme family.	2010 Dec	20616209
Fasting and fat-loading tests provide pathophysiological insight into short-chain acyl-coenzyme a dehydrogenase deficiency.	2010 Jan	19800078
Flavin adenine dinucleotide status and the effects of high-dose riboflavin treatment in short-chain acyl-CoA dehydrogenase deficiency.	2010 Mar	19952864
Silent increase of urinary ethylmalonic acid is an indicator of nonspecific brain dysfunction.	2010 May	20187168
6-[Bis(ethoxycarbonyl)methyl]-6-deoxy-1,2;3,4-di-O-isopropyl-idene-d-galacto-pyran-ose.	2010 Nov 17	21589509
A systems biology approach uncovers cellular strategies used by Methylobacterium extorquens AM1 during the switch from multi- to single-carbon growth.	2010 Nov 24	21124828
Pathogenesis and treatment of mitochondrial myopathies: recent advances.	2010 Oct	21314015
Clinical aspects of short-chain acyl-CoA dehydrogenase deficiency.	2010 Oct	20429031

Patents

Substance Class	Chemical Created by `admin` on `Fri Dec 15 19:43:22 GMT 2023` Edited by `admin` on `Fri Dec 15 19:43:22 GMT 2023`
Record UNII	432NF49DFG
Record Status	`Validated (UNII)`
Record Version

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Name

Type

Language

ETHYLMALONIC ACID

Systematic Name

English

NSC-96615

Code

English

Code System Code Type Description

ECHA (EC/EINECS)

210-007-2