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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: 132 relevant articles (0 outcomes, 3 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. Barschak, Alethéa G: 3 articles (03/2008 - 12/2006)
4. Deon, Marion: 3 articles (03/2008 - 12/2006)
5. Dutra-Filho, Carlos S: 3 articles (03/2008 - 12/2006)
6. Sitta, Angela: 3 articles (03/2008 - 12/2006)
7. Vargas, Carmen R: 3 articles (03/2008 - 12/2006)
8. Vockley, Jerry: 2 articles (06/2022 - 01/2016)
9. Herman, Mark A: 2 articles (10/2021 - 06/2018)
10. McGarrah, Robert W: 2 articles (10/2021 - 06/2018)

Related Diseases

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

Related Drugs and Biologics

1. Enzymes
2. Glucose (Dextrose)
3. Lipids
4. Branched-Chain Amino Acids
5. Leucine (L-Leucine)
6. Isoleucine (L-Isoleucine)
7. Valine (L-Valine)
8. Proteins (Proteins, Gene)
9. Phosphotransferases (Kinase)
10. Proteasome Endopeptidase Complex (Proteasome)

Related Therapies and Procedures

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