RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58067177
T1 Complex III Deficiency
T2 Syndromes: Rapid Recognition and Perioperative Implications
YR 2006
FD 2006
PB The McGraw-Hill Companies
PP New York, NY
SN 9780071354554
LK accessanesthesiology.mhmedical.com/content.aspx?aid=58067177
RD 2024/04/25
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.