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3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) (Alpha-Keto Acid Dehydrogenase)

A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.
Also Known As:
Alpha-Keto Acid Dehydrogenase; Branched Chain Ketoacid Dehydrogenase; 2-Oxoisocaproate Dehydrogenase; BCKA Decarboxylase; Branched Chain Alpha-Keto Acid Decarboxylase; Branched-Chain 2-Oxo Acid Dehydrogenase; Branched-Chain Keto Acid Dehydrogenase; Branched-Chain Oxo-Acid Dehydrogenase; 2 Oxoisocaproate Dehydrogenase; Acid Dehydrogenase, Alpha-Keto; Alpha Keto Acid Dehydrogenase; Branched Chain 2 Oxo Acid Dehydrogenase; Branched Chain Alpha Keto Acid Decarboxylase; Branched Chain Keto Acid Dehydrogenase; Branched Chain Oxo Acid Dehydrogenase; Decarboxylase, BCKA; Dehydrogenase, 2-Oxoisocaproate; Dehydrogenase, Alpha-Keto Acid; Dehydrogenase, Branched-Chain Oxo-Acid; Oxo-Acid Dehydrogenase, Branched-Chain; 2-Oxoisovalerate Dehydrogenase (Lipoamide)
Networked: 106 relevant articles (0 outcomes, 2 trials/studies)

Bio-Agent Context: Research Results

Experts

1. Wajner, Moacir: 4 articles (03/2008 - 06/2005)
2. Lynch, Christopher J: 3 articles (12/2014 - 12/2007)
3. Sitta, Angela: 3 articles (03/2008 - 12/2006)
4. Vargas, Carmen R: 3 articles (03/2008 - 12/2006)
5. Deon, Marion: 3 articles (03/2008 - 12/2006)
6. Dutra-Filho, Carlos S: 3 articles (03/2008 - 12/2006)
7. Barschak, Alethéa G: 3 articles (03/2008 - 12/2006)
8. Shimomura, Yoshiharu: 2 articles (10/2009 - 03/2004)
9. Sato, Yuzo: 2 articles (10/2009 - 03/2004)
10. Nagasaki, Masaru: 2 articles (10/2009 - 03/2004)

Related Diseases

1. Maple Syrup Urine Disease
2. Starvation
3. Biliary Liver Cirrhosis (Primary Biliary Cirrhosis)
4. Acidosis
5. Obesity

Related Drugs and Biologics

1. Branched-Chain Amino Acids
2. Leucine (L-Leucine)
3. Isoleucine (L-Isoleucine)
4. Proteasome Endopeptidase Complex (Proteasome)
5. Ubiquitin
6. Valine (L-Valine)
7. Pyruvic Acid (Pyruvate)
8. Oxidoreductases
9. Autoantibodies
10. 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) (Alpha-Keto Acid Dehydrogenase)

Related Therapies and Procedures

1. Protein-Restricted Diet (Diet, Protein Restricted)
2. Enteral Nutrition (Feeding, Tube)