Reactome | Intermediate 1 binds HP subcomplex to form Intermediate 2

Intermediate 1 binds HP subcomplex to form Intermediate 2

Stable Identifier

R-HSA-6799178

Type

Reaction [binding]

Species

Homo sapiens

Compartment

mitochondrial inner membrane

ReviewStatus

5/5

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Intermediate 1 binds HP subcomplex to form Intermediate 2

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The hydrophobic protein fraction (HP) is assembled with NDUFA3, 8, 9 and 13 amongst many others and anchored to the inner mitochondrial membrane by Intermediate 1 assembly factors NDUFAF3 (C3orf60), NDUFAF4 (C6orf66) and TIMMDC1 (C3orf1) to form Intermediate 2 (Mckenzie & Ryan 2010, Andrews et al. 2013). LYR motif-containing protein 2 (LYRM2) interacts with Complex I pre-assembly complexes via the subunit NDUFAB1, subunit of HP, in a transient manner. LYRM2 knockout cell lines of HEK293T cells showed reduced levels of a subset of complex I subunits and assembly factors (Dibley et al., 2019). We chose to annotate it as a requirement for this reaction, but the exact mechanism of LYRM2 is still unclear.

Literature References

PubMed ID	Title	Journal	Year
20552642	Assembly factors of human mitochondrial complex I and their defects in disease McKenzie, M, Ryan, MT	IUBMB Life	2010
24191001	Assembly factors for the membrane arm of human complex I Andrews, B, Carroll, J, Ding, S, Fearnley, IM, Walker, JE	Proc. Natl. Acad. Sci. U.S.A.	2013
31666358	The Mitochondrial Acyl-carrier Protein Interaction Network Highlights Important Roles for LYRM Family Members in Complex I and Mitoribosome Assembly Dibley, MG, Formosa, LE, Lyu, B, Reljic, B, McGann, D, Muellner-Wong, L, Kraus, F, Sharpe, AJ, Stroud, DA, Ryan, MT	Mol Cell Proteomics	2020