TY - CHAP M1 - Book, Section TI - Complex III Deficiency A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Marciniak, Bruno A1 - Dalens, Bernard J. PY - 2006 T2 - Syndromes: Rapid Recognition and Perioperative Implications AB - Complex III is located within the inner membrane of the mitochondria and is the second enzyme in the electron transport chain of the oxidative phosphorylation process. The ubiquinol cytochrome c reductase catalyses the electron transfer from succinate and nicotinamide adenine dinucleotide-linked dehydrogenases to cytochrome c within the respiratory chain located within the inner membrane of the mitochondria. The clinical features usually include progressive ataxia, predominantly proximal muscle weakness, areflexia, extensor plantar responses, dementia, and concomitant nonspecific myopathic and neuropathic changes in muscle. External ophthalmoplegia, ptosis and cardiomyopathy are often present. The most frequent clinical condition is Leber Myopathy. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/12/13 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=58067177 ER -