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ethyl malonic acid
propanedioic acid, ethyl- (9CI)

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Name:2-ethylpropanedioic acid
CAS Number: 601-75-2Picture of molecule3D/inchi
ECHA EINECS - REACH Pre-Reg:210-007-2
Nikkaji Web:J134.569I
Beilstein Number:774334
XlogP3-AA:0.40 (est)
Molecular Weight:132.11556000
Formula:C5 H8 O4
NMR Predictor:Predict (works with chrome, Edge or firefox)
Category:information only not used for fragrances or flavors
US / EU / FDA / JECFA / FEMA / FLAVIS / Scholar / Patent Information:
Google Scholar:Search
Google Books:Search
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Google Scholar: with word "odor"Search
Google Patents:Search
US Patents:Search
EU Patents:Search
Pubchem Patents:Search
Physical Properties:
Assay: 95.00 to 100.00
Food Chemicals Codex Listed: No
Soluble in:
 water, 7.12E+05 mg/L @ 25 °C (exp)
Organoleptic Properties:
Odor and/or flavor descriptions from others (if found).
Cosmetic Information:
None found
BOC Sciences
For experimental / research use only.
Ethylmalonic Acid
Santa Cruz Biotechnology
For experimental / research use only.
Ethylmalonic Acid ≥97%
Sigma-Aldrich: Aldrich
For experimental / research use only.
Ethylmalonic Acid 97%
For experimental / research use only.
Ethylmalonic Acid >98.0%(T)
Safety Information:
Preferred SDS: View
Hazards identification
Classification of the substance or mixture
GHS Classification in accordance with 29 CFR 1910 (OSHA HCS)
None found.
GHS Label elements, including precautionary statements
Hazard statement(s)
None found.
Precautionary statement(s)
None found.
Oral/Parenteral Toxicity:
Not determined
Dermal Toxicity:
Not determined
Inhalation Toxicity:
Not determined
Safety in Use Information:
information only not used for fragrances or flavors
Recommendation for ethyl malonic acid usage levels up to:
 not for fragrance use.
Recommendation for ethyl malonic acid flavor usage levels up to:
 not for flavor use.
Safety References:
EPI System: View
AIDS Citations:Search
Cancer Citations:Search
Toxicology Citations:Search
EPA ACToR:Toxicology Data
EPA Substance Registry Services (SRS):Registry
Laboratory Chemical Safety Summary :11756
National Institute of Allergy and Infectious Diseases:Data
WGK Germany:3
2-ethylpropanedioic acid
 2-ethylpropanedioic acid
NIST Chemistry WebBook:Search Inchi
Pubchem (cid):11756
Pubchem (cas):601-75-2
Other Information:
(IUPAC):Atomic Weights of the Elements 2011 (pdf)
Videos:The Periodic Table of Videos
tgsc:Atomic Weights use for this web site
(IUPAC):Periodic Table of the Elements
Metabolomics Database:Search
HMDB (The Human Metabolome Database):HMDB00622
Export Tariff Code:2917.19.7050
Potential Blenders and core components note
None Found
Potential Uses:
None Found
Occurrence (nature, food, other):note
 not found in nature
alpha-carboxybutyric acid
 ethylmalonic acid
2-ethylpropanedioic acid
 malonic acid, ethyl- (8CI)
1,1-propanedicarboxylic acid
 propanedioic acid, ethyl- (9CI)


PubMed:Short-chain acyl-CoA dehydrogenase deficiency: from gene to cell pathology and possible disease mechanisms.
PubMed:Identification and Quantitation of Malonic Acid Biomarkers of In-Born Error Metabolism by Targeted Metabolomics.
PubMed:Compound heterozygous mutations of ACADS gene in newborn with short chain acyl-CoA dehydrogenase deficiency: case report and literatures review.
PubMed:Short-Term Pretreatment of Sub-Inhibitory Concentrations of Gentamycin Inhibits the Swarming Motility of Escherichia Coli by Down-Regulating the Succinate Dehydrogenase Gene.
PubMed:Novel and Recurrent ACADS Mutations and Clinical Manifestations Observed in Korean Patients with Short-chain Acyl-coenzyme a Dehydrogenase Deficiency.
PubMed:Clinical relevance of short-chain acyl-CoA dehydrogenase (SCAD) deficiency: Exploring the role of new variants including the first SCAD-disease-causing allele carrying a synonymous mutation.
PubMed:The role of previously unmeasured organic acids in the pathogenesis of severe malaria.
PubMed:A case report of short-chain acyl-CoA dehydrogenase deficiency (SCADD).
PubMed:Brain and muscle redox imbalance elicited by acute ethylmalonic acid administration.
PubMed:Disturbance of energy and redox homeostasis and reduction of Na+,K+-ATPase activity provoked by in vivo intracerebral administration of ethylmalonic acid to young rats.
PubMed:Effects of a dietary sweetener on growth performance and health of stressed beef calves and on diet digestibility and plasma and urinary metabolite concentrations of healthy calves.
PubMed:Ethylmalonic acid induces permeability transition in isolated brain mitochondria.
PubMed:Liquid chromatographic-mass spectrometric method for simultaneous determination of small organic acids potentially contributing to acidosis in severe malaria.
PubMed:A Patient with Congenital Generalized Lipodystrophy Due To a Novel Mutation in BSCL2: Indications for Secondary Mitochondrial Dysfunction.
PubMed:Ethylmalonic acid modulates Na+, K(+)-ATPase activity and mRNA levels in rat cerebral cortex.
PubMed:First case report of short-chain acyl-CoA dehydrogenase deficiency in China.
PubMed:Cannabinoid receptor antagonist-induced striated muscle toxicity and ethylmalonic-adipic aciduria in beagle dogs.
PubMed:An infant with ethylmalonic encephalopathy masquerading as a hematologic disorder.
PubMed:Arabidopsis ETHE1 encodes a sulfur dioxygenase that is essential for embryo and endosperm development.
PubMed:A Rare Case of Short-Chain Acyl-COA Dehydrogenase Deficiency: The Apparent Rarity of the Disorder Results in Under Diagnosis.
PubMed:Ethylmalonic encephalopathy associated with crescentic glomerulonephritis.
PubMed:Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California.
PubMed:Ethylmalonic acid impairs brain mitochondrial succinate and malate transport.
PubMed:Synthesis, structural analysis, and magnetic properties of ethylmalonate-manganese(II) complexes.
PubMed:Decreased oxidative phosphorylation and PGAM deficiency in horses suffering from atypical myopathy associated with acquired MADD.
PubMed:Clinical and neuropathological picture of ethylmalonic aciduria - diagnostic dilemma.
PubMed:Mutations of ACADS gene associated with short-chain acyl-coenzyme A dehydrogenase deficiency.
PubMed:Multiple sources of metabolic disturbance in ETHE1-related ethylmalonic encephalopathy.
PubMed:Streamlined pentafluorophenylpropyl column liquid chromatography-tandem quadrupole mass spectrometry and global (13)C-labeled internal standards improve performance for quantitative metabolomics in bacteria.
PubMed:Combined treatment with oral metronidazole and N-acetylcysteine is effective in ethylmalonic encephalopathy.
PubMed:Ethylmalonic encephalopathy: application of improved biochemical and molecular diagnostic approaches.
PubMed:Antioxidant dysfunction: potential risk for neurotoxicity in ethylmalonic aciduria.
PubMed:Clinical aspects of short-chain acyl-CoA dehydrogenase deficiency.
PubMed:Silent increase of urinary ethylmalonic acid is an indicator of nonspecific brain dysfunction.
PubMed:Flavin adenine dinucleotide status and the effects of high-dose riboflavin treatment in short-chain acyl-CoA dehydrogenase deficiency.
PubMed:Fasting and fat-loading tests provide pathophysiological insight into short-chain acyl-coenzyme a dehydrogenase deficiency.
PubMed:Promotion of lipid and protein oxidative damage in rat brain by ethylmalonic acid.
PubMed:Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy.
PubMed:Short-chain acyl-coenzyme A dehydrogenase deficiency.
PubMed:Chronic early postnatal administration of ethylmalonic acid to rats causes behavioral deficit.
PubMed:[Short-chain acyl-CoA dehydrogenase deficiency (SCADD): relatively high prevalence in the Netherlands and strongly variable fenotype; neonatal screening not indicated].
PubMed:Severe infantile hypotonia with ethylmalonic aciduria: case report.
PubMed: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.
PubMed:Acquired multiple Acyl-CoA dehydrogenase deficiency in 10 horses with atypical myopathy.
PubMed:A new case of short-chain acyl-CoA dehydrogenase deficiency: clinical, biochemical, genetic and (1)H-NMR spectroscopic studies.
PubMed:Ethylmalonic encephalopathy: clinical and biochemical observations.
PubMed:Lessons to learn from rare inborn errors of metabolism.
PubMed:Equine biochemical multiple acyl-CoA dehydrogenase deficiency (MADD) as a cause of rhabdomyolysis.
PubMed:Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency.
PubMed:Single-nucleotide variations in the genes encoding the mitochondrial Hsp60/Hsp10 chaperone system and their disease-causing potential.
PubMed:Clinical, biochemical, and genetic heterogeneity in short-chain acyl-coenzyme A dehydrogenase deficiency.
PubMed:Persistent increase of plasma butyryl/isobutyrylcarnitine concentrations as marker of SCAD defect and ethylmalonic encephalopathy.
PubMed:A case of ethylmalonic encephalopathy with atypical clinical and biochemical presentation.
PubMed:Effect of in vivo administration of ethylmalonic acid on energy metabolism in rat tissues.
PubMed:Inhibition of the electron transport chain and creatine kinase activity by ethylmalonic acid in human skeletal muscle.
PubMed: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.
PubMed:Ethylmalonic encephalopathy-report of two cases.
PubMed:ETHE1 mutations are specific to ethylmalonic encephalopathy.
PubMed:Perioperative management of a child with short-chain acyl-CoA dehydrogenase deficiency.
PubMed:Acyl-CoA dehydrogenase deficiency: varieties with neurological involvement.
PubMed:Recent developments and new applications of tandem mass spectrometry in newborn screening.
PubMed:The role of methionine in ethylmalonic encephalopathy with petechiae.
PubMed:Ethylmalonic encephalopathy is caused by mutations in ETHE1, a gene encoding a mitochondrial matrix protein.
PubMed:[Late-onset riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II)].
PubMed:Short-chain Acyl-CoA dehydrogenase deficiency: studies in a large family adding to the complexity of the disorder.
PubMed:A comparison of in vitro acylcarnitine profiling methods for the diagnosis of classical and variant short chain acyl-CoA dehydrogenase deficiency.
PubMed:Mutation analysis of copper transporter genes in patients with ethylmalonic encephalopathy, mitochondriopathies and copper deficiency phenotypes.
PubMed:Recurrent vomiting and ethylmalonic aciduria associated with rare mutations of the short-chain acyl-CoA dehydrogenase gene.
PubMed:Ethylmalonic acid inhibits mitochondrial creatine kinase activity from cerebral cortex of young rats in vitro.
PubMed:Inhibition of creatine kinase activity in vitro by ethylmalonic acid in cerebral cortex of young rats.
PubMed:Ethylmalonic encephalopathy: further clinical and neuroradiological characterization.
PubMed:Purification and characterization of two polymorphic variants of short chain acyl-CoA dehydrogenase reveal reduction of catalytic activity and stability of the Gly185Ser enzyme.
PubMed:Hypoglycaemia and elevated urine ethylmalonic acid in a child homozygous for the short-chain acyl-CoA dehydrogenase 625G > A gene variation.
PubMed:Therapeutic trial in the first three Asian cases of ethylmalonic encephalopathy: response to riboflavin.
PubMed:ENDOR studies of VO2+: probing protein-metal ion interactions in nephrocalcin.
PubMed:Efficient deuterium-carbon REDOR NMR spectroscopy.
PubMed:Artefactual pyruvate and 2-oxobutyrate produced by trimethylsilylation of methylmalonic and ethylmalonic acids in the presence of oxygen.
PubMed:Urinary organic acid screening in children with developmental language delay.
PubMed:Clinical and brain 18fluoro-2-deoxyglucose positron emission tomographic findings in ethylmalonic aciduria, a progressive neurometabolic disease.
PubMed:Adsorption of small hydroxy acids on glass: a pitfall in quantitative urinary organic acid analysis by GC-MS.
PubMed:A polymorphic variant in the human electron transfer flavoprotein alpha-chain (alpha-T171) displays decreased thermal stability and is overrepresented in very-long-chain acyl-CoA dehydrogenase-deficient patients with mild childhood presentation.
PubMed:Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa.
PubMed:Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism.
PubMed:[Malonic, methylmalonic and ethylmalonic acid semialdehyde dehydrogenase deficiency].
PubMed:Identification of four new mutations in the short-chain acyl-CoA dehydrogenase (SCAD) gene in two patients: one of the variant alleles, 511C-->T, is present at an unexpectedly high frequency in the general population, as was the case for 625G-->A, together conferring susceptibility to ethylmalonic aciduria.
PubMed:Mild or absent clinical signs in twin sisters with short-chain acyl-CoA dehydrogenase deficiency.
PubMed:Central nervous system malformations in ethylmalonic encephalopathy.
PubMed:Structural organization of the human short-chain acyl-CoA dehydrogenase gene.
PubMed:Syndrome of encephalopathy, petechiae, and ethylmalonic aciduria.
PubMed:Stridor as the major presenting symptom in riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency.
PubMed:Ethylmalonic adipic aciduria--a treatable hepatomuscular disorder in two adult brothers.
PubMed:Ethylmalonic aciduria is associated with an amino acid variant of short chain acyl-coenzyme A dehydrogenase.
PubMed:Indirect ultraviolet detection of biologically relevant organic acids by capillary electrophoresis.
PubMed:Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency.
PubMed:Ethylmalonic aciduria: an organic acidemia with CNS involvement and vasculopathy.
PubMed:Amino acid polymorphism (Gly209Ser) in the ACADS gene.
PubMed:Determination of methylmalonic acid by high-performance liquid chromatography.
PubMed:A new syndrome with ethylmalonic aciduria and normal fatty acid oxidation in fibroblasts.
PubMed:Muscle cytochrome c oxidase deficiency in two Italian patients with ethylmalonic aciduria and peculiar clinical phenotype.
PubMed:A new case of short-chain acyl-CoA dehydrogenase deficiency with isolated ethylmalonic aciduria.
PubMed:Breeding experiments to combine the X-linked sparse-fur (spf) mutation with the autosomal recessive BALB/cByJ strain: testing the biochemical phenotype of double-mutant mice as a model for ammonia: fatty acyl CoA synergism.
PubMed:Eating disorder in a 6-year-old Asian girl with ethyl malonic adipic aciduria.
PubMed:[Synthesis and pharmacological activity of ethyl N-acetic and N-malonic esters of heterocyclic derivatives].
PubMed:Combined malonic, methylmalonic and ethylmalonic acid semialdehyde dehydrogenase deficiencies: an inborn error of beta-alanine, L-valine and L-alloisoleucine metabolism?
PubMed:Ethylmalonic aciduria associated with progressive neurological disease and partial cytochrome c oxidase deficiency.
PubMed:New clinical phenotype of branched-chain acyl-CoA oxidation defect.
PubMed:Ethylmalonic/adipic aciduria: effects of oral medium-chain triglycerides, carnitine, and glycine on urinary excretion of organic acids, acylcarnitines, and acylglycines.
PubMed:Possible deleterious effect of L-carnitine supplementation in a patient with mild multiple acyl-CoA dehydrogenation deficiency (ethylmalonic-adipic aciduria).
PubMed:Riboflavin responsive ethylmalonic-adipic aciduria in a 9-month-old boy with liver cirrhosis, myopathy and encephalopathy.
PubMed:Valproate-induced coma with ketosis and carnitine insufficiency.
PubMed:Organic acid and acylcarnitine profiles of glutaric aciduria type I.
PubMed:Sudden infant death syndrome and inherited disorders of fatty acid beta-oxidation.
PubMed:Progressive fatal pancytopenia, psychomotor retardation and muscle carnitine deficiency in a child with ethylmalonic aciduria and ethylmalonic acidaemia.
PubMed:Methylmalonic acid in the diagnosis of cobalt deficiency in barley-fed lambs.
PubMed:Organic aciduria and butyryl CoA dehydrogenase deficiency in BALB/cByJ mice.
PubMed:Short-chain acyl-coenzyme A dehydrogenase deficiency in mice.
PubMed:Sudden infant death syndrome and multiple acyl-coenzyme A dehydrogenase deficiency, ethylmalonic-adipic aciduria, or systemic carnitine deficiency.
PubMed:Short-chain acyl-coenzyme A dehydrogenase deficiency. Clinical and biochemical studies in two patients.
PubMed:Biosynthesis of electron transfer flavoprotein in a cell-free system and in cultured human fibroblasts. Defect in the alpha subunit synthesis is a primary lesion in glutaric aciduria type II.
PubMed:Riboflavin responsive multiple acyl-CoA dehydrogenation deficiency. Assessment of 3 years of riboflavin treatment.
PubMed:The multiple acyl-coenzyme A dehydrogenation disorders, glutaric aciduria type II and ethylmalonic-adipic aciduria. Mitochondrial fatty acid oxidation, acyl-coenzyme A dehydrogenase, and electron transfer flavoprotein activities in fibroblasts.
PubMed:Model for calcium binding to gamma-carboxyglutamic acid residues of proteins: crystal structure of calcium alpha-ethylmalonate.
PubMed:Riboflavin-responsive ethylmalonic-adipic aciduria.
PubMed:Multiple acyl-CoA dehydrogenase deficiency: a neonatal onset case responsive to treatment.
PubMed:Electron-transferring flavoprotein deficiency in the multiple acyl-CoA dehydrogenation disorders, glutaric aciduria type II and ethylmalonic--adipic aciduria.
PubMed:Metabolism of ethylmalonate to mesaconate in the rat. Evidence for trans-dehydrogenation of methylsuccinate.
PubMed:The urinary excretion of ethylmalonic acid: what level requires further attention?
PubMed:C6-C10-dicarboxylic aciduria: investigations of a patient with riboflavin responsive multiple acyl-CoA dehydrogenation defects.
PubMed:Recurrent hypoglycemia associated with glutaric aciduria type II in an adult.
PubMed:Metabolism in rats in vivo of RS-2-methylbutyrate and n-butyrate labeled with stable isotopes at various positions. Mechanism of biosynthesis and degradation of ethylmalonyl semialdehyde and ethylmalonic acid.
PubMed:Investigation of the mechanism of the methylmalonyl-CoA mutase reaction with the substrate analogue: ethylmalonyl-CoA.
PubMed:Studies on the biosynthesis of 16-membered macrolide antibiotics using carbon-13 nuclear magnetic resonance spectroscopy.
PubMed:Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites.
PubMed:Identification of ethylmalonic acid in urine of two patients with the vomitting sickness of Jamaica.
PubMed:A simplified and rapid quantitative assay for propionic and methylmalonic acids in urine.
Ethylmalonic acid is identified in the urine of patients with short-chain acyl-coenzyme A dehydrogenase deficiency, which is a fatty acid metabolism disorder. [HMDB]
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