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alpha-ketoisovalerate

RN given refers to parent cpd
Also Known As:
2-ketoisovalerate; 2-oxoisovalerate; 3-methyl-2-oxobutanoate; 3-methyl-2-oxobutyrate; alpha-keto-isovaleric acid; alpha-ketoisopentanoic acid; alpha-ketoisovalerate, calcium salt; alpha-ketoisovalerate, sodium salt; alpha-ketoisovaleric acid; alpha-ketovaline; alpha-oxoisovalerate; calcium ketovaline
Networked: 14 relevant articles (0 outcomes, 1 trials/studies)

Bio-Agent Context: Research Results

Experts

1. Wajner, Moacir: 3 articles (09/2007 - 06/2005)
2. Funchal, Cláudia: 2 articles (09/2007 - 02/2006)
3. Pessoa-Pureur, Regina: 2 articles (09/2007 - 02/2006)
4. Kand'ár, Roman: 1 article (01/2009)
5. Jirosová, Jana: 1 article (01/2009)
6. Záková, Pavla: 1 article (01/2009)
7. Sladká, Michaela: 1 article (01/2009)
8. Quincozes dos Santos, André: 1 article (09/2007)
9. Tramontina, Francine: 1 article (09/2007)
10. Gonçalves, Carlos Alberto: 1 article (09/2007)

Related Diseases

1. Hyperphosphatemia
2. Maple Syrup Urine Disease
05/01/1982 - "Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate."
09/01/1975 - "Cultured cells of normal individuals and nine patients with different clinical pictures of maple syrup urine disease (MSUD) are studied with both alpha-ketoisocaproic acid (2-oxo-4-methylpentanoic acid (KIC)) and alpha-ketoisovaleric acid (2-oxo-3-methylbutanoic acid (KIVA)) as substrates. "
09/15/2007 - "Accumulation of the branched-chain alpha-keto acids (BCKA), alpha-ketoisocaproic acid (KIC), alpha-keto-beta-methylvaleric acid (KMV) and alpha-ketoisovaleric acid (KIV) and their respective branched-chain alpha-amino acids (BCAA) occurs in tissues and biological fluids of patients affected by the neurometabolic disorder maple syrup urine disease (MSUD). "
02/01/2006 - "Accumulation of the branched-chain alpha-keto acids (BCKA), alpha-ketoisocaproic acid (KIC), alpha-keto-beta-methylvaleric acid (KMV), and alpha-ketoisovaleric acid (KIV) and their respective branched-chain alpha-amino acids (BCAA) in tissues and biological fluids is the biochemical hallmark of patients affected by the neurometabolic disorder known as maple syrup urine disease (MSUD). "
06/01/2005 - "Maple syrup urine disease (MSUD) is an inherited neurometabolic disorder caused by deficiency of branched-chain alpha-keto acid dehydrogenase complex activity which leads to tissue accumulation of the branched-chain alpha-keto acids (BCKAs) alpha-ketoisocaproic acid (KIC), alpha-ketoisovaleric acid (KIV) and alpha-keto-beta-methylvaleric acid (KMV) and their respective amino acids. "
3. Tyrosinemias (Tyrosinemia)
4. Seizures (Seizure)
5. Phenylketonurias (Phenylketonuria)

Related Drugs and Biologics

1. Amino Acids
2. Salts
3. Calcium
4. Aluminum
5. alpha-ketoisocaproic acid
6. Keto Acids
7. alpha-keto-beta-methylvaleric acid (KMVA)
8. 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) (Alpha-Keto Acid Dehydrogenase)
9. Pyruvic Acid (Pyruvate)
10. Valine (L-Valine)