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Beta-Methylcrotonylglycinuria

Known as: Beta-methylcrotonyglycinuria (Types I or II) 
A very rare metabolic disorder characterized by an inborn error in the leucine degradation pathway. Patients excrete large amounts of beta-methyl… 
National Institutes of Health

Papers overview

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Highly Cited
1974
Highly Cited
1974
Studies on the urinary acidic metabolites excreted by patients with beta-methylcrotonylglycinuria, propionic acidaemia and methylmalonic acidaemia, using gas-liquid chromatography and mass…
1974
1974
A method for the determination of volatile organic acids in aqueous solutions and urine, and the results obtained in propionic acidaemia, beta-methylcrotonylglycinuria and methylmalonic aciduria.
1974
1974
Comparative GC/MS studies on the urinary acidic metabolites in beta-methylcrotonylglycinuria propionic acidaemia and methylmalonic acidaemia.
1974
1974
Mitochondrial inclusions in fibroblast culture from a patient with β-methylcrotonylglycinuria
Electron microscopy of fibroblasts grown from a patient with biotin responsive β-methylcrotonylglycinuria revealed unusual… 
1973
1973
Child with a defect in leucine metabolism associated with beta-hydroxyisovaleric aciduria and beta-methylcrotonylglycinuria.
Three inborn errors of the leucine degradation pathway are known, maple syrup urine disease, isovaleric acidaemia, and ,B… 
1973
1973
Evidence for the enzymic defect in beta-methylcrotonylglycinuria.
1971
1971
Biotin-responsive beta-methylcrotonylglycinuria.
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