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2-Methylacetoacetate

Known as: 2-Methyl-3-Oxobutyrate 
National Institutes of Health

Related topics

Related topics

2 relations
alpha-ketoisovalerate

Broader (1)

methyl acetoacetate

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2016
2016
Japanese Male Siblings with 2-Methyl-3-Hydroxybutyryl-CoA Dehydrogenase Deficiency (HSD10 Disease) Without Neurological Regression.
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (HSD10 disease) is a rare X-linked disorder caused by a mutation in the… 
2011
2011
Myoglobin-H2O2 catalyzes the oxidation of β-ketoacids to α-dicarbonyls: mechanism and implications in ketosis.
2008
2008
Biochemical profiling in two siblings with mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency.
We report the biochemical profiling in two siblings with mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency. Organic… 
2007
2007
[Mitochondrial acetoacetyl-CoA thiolase deficiency: neonatal onset].
We present the case of a 4-day-old newborn with serious dehydration, polypnea, hypertonus and lethargy. Blood analysis showed… 
2004
2004
Peroxynitrite-initiated oxidation of acetoacetate and 2-methylacetoacetate esters by oxygen: potential sources of reactive intermediates in keto acidoses.
Oxidative stress is believed to play a role in the pathogenesis of several diseases, including diabetes and inborn errors of… 
1996
1996
Metabolic studies in twin brothers with 2-methylacetoacetyl-CoA thiolase deficiency.
1986
1986
Spin-echo and 2-dimensional 1H nuclear magnetic resonance studies on urinary metabolites from patients with 2-methylacetoacetyl CoA thiolase deficiency.
1980
1980
Metabolism of L-beta-lysine by a Pseudomonas: purification and properties of 3-keto-6-acetamidohexanoate cleavage enzyme.
1979
1979
Propionic acidaemia presenting with pancytopaenia in infancy
A 2-month-old infant presented with vomiting, lethargy and pancytopaenia. She was found to have propionic acidaemia, and the… 
1977
1977
2-methylacetoacetate reductase and possible propionyl coenzyme A condensing enzyme activity in branched chain volatile fatty acid synthesis by Ascaris lumbricoides.
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