Unknown peptidase cleaves UQCRFS1 subunit

Stable Identifier
R-HSA-9906017
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial inner membrane
ReviewStatus
5/5
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Unknown peptidase cleaves UQCRFS1 subunit
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Proteolytic processing is necessary for the correct insertion of UQCRFS1 in the complex III dimer. An unknown peptidase cleaves the N-terminal 78 amino acids of UQCRFS1, producing several unspecified N-terminal fragments that remain in the complex until cleared by TTC19. Possible candidates for the peptidase are the UQCRC1 and UQCRC2 subunits (as hinted at in the cattle model) or the SPY complex (Bottani et al., 2017; Fernandez-Vizarra & Zeviani, 2018).
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

Dogan, SA, Fearnley, IM, Giordano, C, Harbour, ME, Cerutti, R, D'Amati, G, Ravaglia, S, Viscomi, C, Fernandez-Vizarra, E, Bottani, E, Zeviani, M

Mol Cell 2017
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Output
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as an event of
Catalyst Activity

endopeptidase activity of Unidentified protease [mitochondrial inner membrane]

Physical Entity
Unidentified protease [mitochondrial inner membrane] (Homo sapiens)
Activity
endopeptidase activity (GO:0004175)
Authored
Stephan, R  (2024-03-29)
Reviewed
Hill, DP  (2024-05-16)
Created
Stephan, R  (2024-03-29)
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