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ethylmalonic acid

Known as: 1,1-Propanedicarboxylic acid, alpha-carboxybutyric acid, propanedioic acid, ethyl- 
National Institutes of Health

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Malonates

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ethylmalonate

Papers overview

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2014
2014
Ethylmalonic Acid Induces Permeability Transition in Isolated Brain Mitochondria
Predominant accumulation of ethylmalonic acid (EMA) in tissues and biological fluids is a characteristic of patients affected by… 
2010
2010
Fasting and fat-loading tests provide pathophysiological insight into short-chain acyl-coenzyme a dehydrogenase deficiency.
2004
2004
The role of methionine in ethylmalonic encephalopathy with petechiae.
BACKGROUND Among patients with ethylmalonic aciduria, a subgroup with encephalopathy, petechial skin lesions, and often death in… 
2004
2004
Brain mitochondrial impairment in ethylmalonic encephalopathy
Sirs: Ethylmalonic encephalopathy (EE) is a rare autosomal recessive metabolic disorder that is clinically characterized by… 
2003
2003
Ethylmalonic Acid Inhibits Mitochondrial Creatine Kinase Activity from Cerebral Cortex of Young Rats in Vitro
Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is an inherited metabolic disorder biochemically characterized by tissue… 
1998
1998
Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism.
1993
1993
A new case of short-chain acyl-CoA dehydrogenase deficiency with isolated ethylmalonic aciduria
A 28-month-old Turkish girl presented with recurrent bronchopneumonia and severe muscular hypotonia. Urinary excretion of… 
1993
1993
Ethylmalonic aciduria associated with progressive neurological disease and partial cytochromec oxidase deficiency
Persistent excretion of elevated amounts of ethylmalonic acid (EMA) with or without concomitant acylglycinuria is a relatively… 
Highly Cited
1979
Highly Cited
1979
Recurrent hypoglycemia associated with glutaric aciduria type II in an adult.
Repeated episodes of hypoglycemia accompanied by elevated serum concentrations of free fatty acid without ketosis, fatty… 
Highly Cited
1976
Highly Cited
1976
Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites.
1. Normal human urine contains small amounts (less than 4 mg/g of creatinine) of 2-ethylhydracrylic acid, formed, we believe, by… 
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