TY - CHAP M1 - Book, Section TI - Complex III Deficiency A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas Y1 - 2019 N1 - T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Complex III is located within the inner membrane of the mitochondrium and is the second enzyme in the electron transport chain of the oxidative phosphorylation process. The enzyme ubiquinol-cytochrome-c oxidoreductase catalyzes the electron transfer from succinate and nicotinamide adenine dinucleotide linked dehydrogenases to cytochrome c within the respiratory chain. The clinical features usually include dementia, progressive ataxia, predominantly proximal muscle weakness, areflexia, extensor plantar responses, and concomitant nonspecific myo- and neuropathic changes in muscles. External ophthalmoplegia, ptosis, and cardiomyopathy are often present. The most frequently associated clinical condition is ☞Leber Hereditary Optic Neuropathy (LHON). SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164066360 ER -