EC Number   |
General Information |
Reference |
|---|
    6.2.1.5 | evolution |
malate-CoA ligases and succinate-CoA ligases might have the same evolutionary origin |
-, 726747 |
| 6.2.1.5 | evolution |
the succinyl-CoA:acetate CoA-transferase/succinyl-CoA synthetase pathway is encoded by 30 species belonging to 5 different phyla, showing that a diverse range of bacteria encode this pathway. The SCACT/SCS pathway is important for acetate formation in many branches of the tree of life |
-, 762653 |
| 6.2.1.5 | malfunction |
enzyme deficiency induces severe mitochondrial dysfunction including lowered oxidative phosphorylation efficiency, increased mitochondrial superoxide production, and mitochondrial DNA depletion as well as aberrations of mitochondrial fusion and fission proteins, which eventually leads to neuronal stress |
746481 |
| 6.2.1.5 | malfunction |
enzyme mutations cause the encephalomyopathic mitochondrial DNA depletion syndrome with methylmalonic aciduria |
745500 |
| 6.2.1.5 | malfunction |
enzyme mutations result in succinyl-CoA ligase (ATP-forming) or succinyl-CoA synthetase (ADP-forming) deficiency, a mitochondrial tricarboxylic acid cycle disorder |
745783 |
| 6.2.1.5 | malfunction |
in patients with enzyme mutations, muscle histology shows severe lipid accumulation, and a marked type I fibre predominance is found, with an increased variability in fibre diameter. mtDNA depletion in succinate-CoA ligase deficiency |
693488 |
| 6.2.1.5 | malfunction |
inhibition of the enzyme might influence the activity of nucleoside diphosphate kinase inducing an imbalance of nucleotides in the mitochondria and eventually mitochondrial DNA depletion. This may account for the potential liver failure associated with valproate therapy, reported in patients with deficiencies within the mitochondrial DNA replicase system such as polymerase gamma 1 |
728078 |
| 6.2.1.5 | malfunction |
knockdown of SUCLG2 by shRNA in both SUCLA2-related mitochondrial DNA depletion syndrome patient and control fibroblasts results in a significant decrease in mtDNA amount, decreased NDPK and cytochrome c oxidase activities, and a marked growth impairment, phenotype, overview |
714342 |
| 6.2.1.5 | metabolism |
key role of the enzyme in the Krebs cycle |
728078 |
| 6.2.1.5 | metabolism |
the enzyme is involved in fermentation of glucose (succinyl-CoA:acetate CoA-transferase/succinyl-CoA synthetase pathway). This pathway represents a way for bacteria to form acetate from acetyl-CoA and synthesize ATP via substrate-level phosphorylation |
-, 762653 |
