RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164066360
T1 Complex III Deficiency
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accesspediatrics.mhmedical.com/content.aspx?aid=1164066360
RD 2024/04/20
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).