carbonyl cyanide p-trifluoromethoxyphenylhydrazone and Leigh Disease

carbonyl cyanide p-trifluoromethoxyphenylhydrazone has been researched along with Leigh 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.

Leigh Disease: A group of metabolic disorders primarily of infancy characterized by the subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, dysphagia, and lactic acidosis. Pathological features include spongy degeneration of the neuropile of the basal ganglia, thalamus, brain stem, and spinal cord. Patterns of inheritance include X-linked recessive, autosomal recessive, and mitochondrial. Leigh disease has been associated with mutations in genes for the PYRUVATE DEHYDROGENASE COMPLEX; CYTOCHROME-C OXIDASE; ATP synthase subunit 6; and subunits of mitochondrial complex I. (From Menkes, Textbook of Child Neurology, 5th ed, p850).

Research

Studies (1)

Authors

Other Studies

1 other study available for carbonyl cyanide p-trifluoromethoxyphenylhydrazone and Leigh Disease