carbonyl cyanide p-trifluoromethoxyphenylhydrazone has been researched along with Pyruvate Dehydrogenase Complex Deficiency Disease in 1 studies
Carbonyl Cyanide p-Trifluoromethoxyphenylhydrazone: A proton ionophore that is commonly used as an uncoupling agent in biochemical studies.
carbonyl cyanide p-trifluoromethoxyphenylhydrazone : A hydrazone that is hydrazonomalononitrile in which one of the hydrazine hydrogens is substituted by a p-trifluoromethoxyphenyl group.
Pyruvate Dehydrogenase Complex Deficiency Disease: An inherited metabolic disorder caused by deficient enzyme activity in the PYRUVATE DEHYDROGENASE COMPLEX, resulting in deficiency of acetyl CoA and reduced synthesis of acetylcholine. Two clinical forms are recognized: neonatal and juvenile. The neonatal form is a relatively common cause of lactic acidosis in the first weeks of life and may also feature an erythematous rash. The juvenile form presents with lactic acidosis, alopecia, intermittent ATAXIA; SEIZURES; and an erythematous rash. (From J Inherit Metab Dis 1996;19(4):452-62) Autosomal recessive and X-linked forms are caused by mutations in the genes for the three different enzyme components of this multisubunit pyruvate dehydrogenase complex. One of the mutations at Xp22.2-p22.1 in the gene for the E1 alpha component of the complex leads to LEIGH DISEASE.
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 0 (0.00) | 18.7374 |
1990's | 1 (100.00) | 18.2507 |
2000's | 0 (0.00) | 29.6817 |
2010's | 0 (0.00) | 24.3611 |
2020's | 0 (0.00) | 2.80 |
Authors | Studies |
---|---|
Padua, RA | 1 |
Baron, KT | 1 |
Thyagarajan, B | 1 |
Campbell, C | 1 |
Thayer, SA | 1 |
1 other study available for carbonyl cyanide p-trifluoromethoxyphenylhydrazone and Pyruvate Dehydrogenase Complex Deficiency Disease
Article | Year |
---|---|
Reduced Ca2+ uptake by mitochondria in pyruvate dehydrogenase-deficient human diploid fibroblasts.
Topics: Aequorin; Apoproteins; Bradykinin; Calcium; Calcium-Binding Proteins; Carbonyl Cyanide p-Trifluorome | 1998 |