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2-methyl-3-hydroxybutyric acid

National Institutes of Health

Papers overview

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2014
2014
Introduction: Mitochondrial acetoacetyl-CoA thiolase (3-oxothiolase) deficiency is a rare metabolic disorder involving ketone… 
1990
1990
ABSTRACT: Cultured fibroblasts from 13 patients with organic aciduria suggesting 3-oxothiolase deficiency were studied by… 
1987
1987
2-Methyl-3-hydroxybutyric aciduria (McKusick 20375) is a disorder of isoleucine metabolism characterized by urinary excretion of… 
1987
1987
[3-Ketothiolase deficiency .(McKusick 20375) is an uncommon inborn error of isoleucine catabolism, characterized by the excretion… 
1987
1987
3-ketothiolase deficiency is an inherited disorder of isoleucine degradation, characterized by excretion of 2-methyl-3… 
1986
1986
Two patients have been studied in whom the activity of the short chain-length-specific mitochondrial 3-ketothiolase was found to… 
1984
1984
1980
1980
By gas chromatographic mass spectrometric analysis, 2-methyl-3-oxovaleric acid, 3-oxovaleric acid, 3-hydroxyvaleric acid and 2… 
1979
1979
An 11-year old girl with spastic paraplegia and mental retardation has suffered from attacks of metabolic acidosis since the age… 
Highly Cited
1978
Highly Cited
1978
A number of previously unrecognized abnormal metabolites have been identified and quantitated in the urine of a patient with an…