TTC19 clears UQCRFS1 fragments from Complex III
Dimer of Late core complex:UQCR10:UQCR11:holo-UQCRFS1(79-274):Fragments → Cytochrome c reductase + 2 Unspecified N-terminal UQCRFS1 fragments
- Reactome
- R-HSA-9906042
- Compartment
- mitochondrial inner membrane
- Rhea
- None recorded
Inputs
- Dimer of Late core complex:UQCR10:UQCR11:holo-UQCRFS1(79-274):FragmentsR-HSA-9906014, mitochondrial inner membrane
Outputs
- Cytochrome c reductaseR-HSA-9866171, mitochondrial inner membrane
- 2 Unspecified N-terminal UQCRFS1 fragmentsR-ALL-9906010, mitochondrial inner membrane
Literature
- TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III Mol Cell, 2017
- Mitochondrial complex III Rieske Fe-S protein processing and assembly Cell Cycle, 2018
- Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies Nat Genet, 2011
- A TTC19 mutation associated with progressive movement disorders and peripheral neuropathy: Case report and systematic review CNS Neurosci Ther, 2024