Reactome | TTC19 clears UQCRFS1 fragments from Complex III

TTC19 clears UQCRFS1 fragments from Complex III

Stable Identifier

R-HSA-9906042

Type

Reaction [uncertain]

Species

Homo sapiens

Compartment

mitochondrial inner membrane

ReviewStatus

5/5

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TTC19 clears UQCRFS1 fragments from Complex III

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N-terminal cleavage fragments of UQCRFS1 are cleared by TTC19, stabilizing the final complex. The process was shown in mouse but the mechanism is unclear (Bottani et al., 2017; Fernandez-Vizarra & Zeviani, 2018). Defects in TTC19 cause mitochondrial complex III deficiency in human (Ghezzi et al., 2011; reviewed in Xuan et al., 2023).

Literature References

PubMed ID	Title	Journal	Year
29243944	Mitochondrial complex III Rieske Fe-S protein processing and assembly Fernandez-Vizarra, E, Zeviani, M	Cell Cycle	2018
28673544	TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III Bottani, E, Cerutti, R, Harbour, ME, Ravaglia, S, Dogan, SA, Giordano, C, Fearnley, IM, D'Amati, G, Viscomi, C, Fernandez-Vizarra, E, Zeviani, M	Mol Cell	2017
21278747	Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies Ghezzi, D, Arzuffi, P, Zordan, M, Da Re, C, Lamperti, C, Benna, C, D'Adamo, P, Diodato, D, Costa, R, Mariotti, C, Uziel, G, Smiderle, C, Zeviani, M	Nat Genet	2011
37927170	A TTC19 mutation associated with progressive movement disorders and peripheral neuropathy: Case report and systematic review Xuan, X, Ruan, J, Wu, C, Gao, Y, Li, L, Lei, X	CNS Neurosci Ther	2024