RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58067164
T1 Complex II 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=58067164
RD 2024/04/24
AB Complex  II  is,  as  in  Complex  I,  caused  by  mutations  in  nDNA.  This  mutation  is  defined  as  a  “direct  hit"  in  the  genes  that  encode  subunits  of  respiratory  chains  complexes.  It  affects  the  enzyme  succinate  CoQ  reductase  which  is  responsible  for  the  transfer  of  electrons  by  the  reduction  of  succinate  to  fumarate  in  the  electron  chain  pathway  (see  Table  C-2).  Deficiency  of  complex  II  is  characterized  by  highly  variable  phenotypic  expression.  The  clinical  features  include  encephalomyopathy,  failure  to  thrive,  severe  developmental  delay,  muscle  hypotonia,  lethargy,  respiratory  failure,  ataxia,  and  myoclonic  seizures.  The  presence  of  lactic  acidosis  is  common.  The  most  frequent  clinical  condition  is  Leigh  syndrome.  See  Table  C-4.