Inhibition of the electron transport chain and creatine kinase activity by ethylmalonic acid in human skeletal muscle

Ethylmalonic aciduria is a common finding in patients affected by short-chain acyl-CoA dehydrogenase (SCAD) deficiency and other diseases characterized by encephalopathy, muscular symptomatology, and lactic acidemia. Considering that the pathophysiological mechanisms of these disorders are practical...

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Veröffentlicht in:	Metabolic brain disease 2006-03, Vol.21 (1), p.11-19
Hauptverfasser:	BARSCHAK, Alethea G, FERREIRA, Gustavo Da C, WAJNER, Moacir, ANDRE, Karina R, SCHUCK, Patricia F, VIEGAS, Carolina M, TONIN, Anelise, DUTRA FILHO, Carlos S, WYSE, Angela T. S, WANNMACHER, Clovis M. D, VARGAS, Carmen R
Format:	Artikel
Sprache:	eng
Schlagworte:	Acidosis, Lactic - metabolism Adenosine Triphosphate - metabolism Adolescent Adult Biological and medical sciences Cerebral circulation. Blood-brain barrier. Choroid plexus. Cerebrospinal fluid. Circumventricular organ. Meninges Creatine Kinase - metabolism Electron Transport Chain Complex Proteins - antagonists & inhibitors Electron Transport Chain Complex Proteins - metabolism Electron Transport Complex I - antagonists & inhibitors Electron Transport Complex I - metabolism Electron Transport Complex II - antagonists & inhibitors Electron Transport Complex II - metabolism Electron Transport Complex III - antagonists & inhibitors Electron Transport Complex III - metabolism Electron Transport Complex IV - antagonists & inhibitors Electron Transport Complex IV - metabolism Energy Metabolism - drug effects Energy Metabolism - physiology Enzyme Activation - drug effects Fundamental and applied biological sciences. Psychology Humans In Vitro Techniques Malonates - pharmacology Medical sciences Muscle, Skeletal - drug effects Muscle, Skeletal - enzymology Neurology Vascular diseases and vascular malformations of the nervous system Vertebrates: nervous system and sense organs
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