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2-methylbutyrylglycine

Known as: Glycine, N-(2-methyl-1-oxobutyl)- 
National Institutes of Health

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2 relations

Broader (1)

Glycine
analogs & derivatives

Papers overview

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2019
2019
Improved clinical outcome following liver transplant in patients with ethylmalonic encephalopathy
Ethylmalonic encephalopathy (EE) is a rapidly progressive autosomal recessive mitochondrial disease caused by biallelic… 
2007
2007
Ethylmalonic encephalopathy: clinical and biochemical observations.
Ethylmalonic encephalopathy (EE) is a rare, recently defined inborn error of metabolism which affects the brain, gastrointestinal… 
2005
2005
2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation.
BACKGROUND Isolated excretion of 2-methylbutyrylglycine (2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase… 
2005
2005
The occurrence of C6–C10-dicarboxylic acids, ethylmalonic acid, 5-hydroxycaproic acid, butyrylglycine, isovalerylglycine, isobutyrylglycine, 2-methylbutyrylglycine and glutaric acid in the urine of…
C6-Clo-Dicarboxylic aciduria may be caused by an inborn error of fatty acid 13-oxidation. In the cases reported the defect has… 
Highly Cited
2000
Highly Cited
2000
2-Methylbutyryl-Coenzyme A Dehydrogenase Deficiency: A New Inborn Error of L-Isoleucine Metabolism
An 4-mo-old male was found to have an isolated increase in 2-methylbutyrylglycine (2-MBG) and 2-methylbutyrylcarnitine (2-MBC) in… 
Highly Cited
1999
Highly Cited
1999
Short-chain acyl–CoA dehydrogenase deficiency
Objective: To determine an underlying genetic defect within the differential diagnosis of congenital multicore myopathy… 
1998
1998
Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism.
1980
1980
Excretion of short-chain N-acylglycines in the urine of a patient with D-glyceric acidemia.
Highly Cited
1978
Highly Cited
1978
Abnormal metabolites of isoleucine in a patient with propionyl-CoA carboxylase deficiency.
A number of previously unrecognized abnormal metabolites have been identified and quantitated in the urine of a patient with an… 
1972
1972
The identification of tiglylglycine in the urine of a child with -methylcrotonylglycinuria.
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