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2-methylacetoacetyl-coenzyme A

Also Known As:

2-methylacetoacetyl-CoA; coenzyme A, 2-methylacetoacetyl-; Coenzyme A, S-(2-methyl-3-oxobutanoate)

Networked: 5 relevant articles (0 outcomes, 1 trials/studies)

Bio-Agent Context: Research Results

Experts

1.	Inahashi, Yuki: 1 article (01/2016)
2.	Kuzuyama, Tomohisa: 1 article (01/2016)
3.	Nakashima, Takuji: 1 article (01/2016)
4.	Palm, Kaia: 1 article (01/2016)
5.	Shiraishi, Taro: 1 article (01/2016)
6.	Takahashi, Yoko: 1 article (01/2016)
7.	Ōmura, Satoshi: 1 article (01/2016)

Related Diseases

1.	Dehydration (Water Stress) 01/01/2016 - "Enzymatic activity studies showed that ThgI catalyses the condensation of acetyl-CoA and methylmalonyl-CoA to 2-methylacetoacetyl-CoA (MAA-CoA), ThgK catalyses NADPH-dependent reduction of MAA-CoA to 3-hydroxy-2-methylbutyryl-CoA (HMB-CoA) and ThgH catalyses the dehydration of HMB-CoA to angelyl-CoA (AN-CoA). "
2.	Coma (Comas) 06/01/1996 - "[Ketoacidotic coma in an infant as the form of onset of a mitochondrial 2-methylacetoacetyl-CoA thiolase deficiency]." 01/01/1995 - "Metabolic coma with ketoacidosis and hyperglycaemia in 2-methylacetoacetyl-CoA thiolase deficiency."
3.	Rare Diseases (Rare Disease) 11/15/1996 - "Through the present report on this rare disease in two siblings, we would like to underline that acylcarnitines can be used in the diagnosis of 2-methylacetoacetyl-CoA thiolase deficiency, a view supported by acylcarnitine profiles further determined in another patient with proven oxothiolase deficiency, adding this pathology to the list of beta-oxidation disorders that may be screened successfully through determination of acylcarnitine profiles in body fluids."
4.	Ketosis 01/01/1995 - "Metabolic coma with ketoacidosis and hyperglycaemia in 2-methylacetoacetyl-CoA thiolase deficiency."
5.	3-Methylglutaconic Aciduria 01/01/1994 - "This report will summarize clinical and metabolite features and enzymological methods available for the diagnosis of the more common defects of branched-chain amino acid metabolism, including isovaleryl-CoA dehydrogenase deficiency, 3-methylcrotonyl-CoA carboxylase deficiency, 3-methylglutaconic aciduria due to 3-methylglutaconyl-CoA hydratase deficiency and other less well characterized defects, 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, and 2-methylacetoacetyl-CoA thiolase deficiency. "

Related Drugs and Biologics

1.	NADP (NADPH)
2.	Coenzyme A (CoA)
3.	Acetyl Coenzyme A (Acetyl-CoA)
4.	12- (4'- azido- 2'- nitrophenoxy)dodecanoyl- coenzyme A
5.	methylmalonyl-coenzyme A (methylmalonyl CoA)
6.	Branched-Chain Amino Acids
7.	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
8.	3-methylcrotonyl CoA carboxylase 1 deficiency
9.	acylcarnitine