This pathway shows an simplified version of the Krebs cycle (for more details see [https://www.wikipathways.org/index.php/Pathway:WP78] ), with 4 genetic diseases related to it.
For succinyl-CoA synthetase deficiencies, relevant metabolic markers are depicted in light green.
Patients with a mutation in the SUCLG1 might present a severe (fatal) form of mitochondrial encephalomyopathy.
This pathway was inspired by Chapter 20 of the book of Blau (ISBN 3642403360 (978-3642403361)).