Disorders caused by deficiency of succinate-CoA ligase

  title={Disorders caused by deficiency of succinate-CoA ligase},
  author={Elsebet Ostergaard},
  journal={Journal of Inherited Metabolic Disease},
Succinate-CoA ligase catalyses the reversible conversion of succinyl-CoA and ADP or GDP to succinate and ATP or GTP. It is a mitochondrial matrix enzyme and at least the ADP-forming enzyme is part of the Krebs cycle. The substrate specificity is determined by the β subunit of succinate-CoA ligase, which is encoded by either SUCLA2 or SUCLG2. In patients with severe hypotonia, deafness and Leigh-like syndrome, mutations have been found in SUCLA2. Mutations have also been reported in SUCLG1… CONTINUE READING
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