Reactome | IP subcomplex binds NDUFAF3, NDUFAF4, TIMMDC1 to form Intermediate 1

IP subcomplex binds NDUFAF3, NDUFAF4, TIMMDC1 to form Intermediate 1

Stable Identifier

R-HSA-6799203

Type

Reaction [binding]

Species

Homo sapiens

Compartment

mitochondrial matrix, mitochondrial inner membrane

ReviewStatus

5/5

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IP subcomplex binds NDUFAF3, NDUFAF4, TIMMDC1 to form Intermediate 1

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Complex I assembly begins with the formation of a 315kDa subcomplex, centred around the core subunits NADH dehydrogenase [ubiquinone] iron-sulfur proteins 2 and 3 (NDUFS2 and NDUFS3) (Mckenzie & Ryan 2010, Mimaki et al. 2012, Andrews et al. 2013). NDUFS2 is thought to be bound to NDUFAF7 (Carilla-Latorre et al. 2010). Defects in NDUFS2 can cause mitochondrial complex I deficiency (MT-C1D; OMIM:252010), causing a wide range of clinical disorders, ranging from lethal neonatal disease to adult-onset neurodegenerative disorders (Loeffen et al. 2001). As an initial part of the 315kDa subcomplex, the subunits NDUFS7, S8 and A9, together with NDUFS2 and S3, form an evolutionarily conserved hydrogenase module as part of the Iron-Sulfur protein fraction (IP) subcomplex (termed Intermediate 1 here) (Mckenzie & Ryan 2010, Andrews et al. 2013).

Literature References

PubMed ID	Title	Journal	Year
20406883	MidA is a putative methyltransferase that is required for mitochondrial complex I function Carilla-Latorre, S, Accari, SL, Calvo-Garrido, J, Annesley, SJ, Smith, PK, Escalante, R, Gallardo, ME, Garesse, R, Graña, O, Fisher, PR, Valencia, A	J. Cell. Sci.	2010
21924235	Understanding mitochondrial complex I assembly in health and disease Wang, X, Ryan, MT, Thorburn, DR, McKenzie, M, Mimaki, M	Biochim. Biophys. Acta	2012
20552642	Assembly factors of human mitochondrial complex I and their defects in disease Ryan, MT, McKenzie, M	IUBMB Life	2010
11220739	Mutations in the complex I NDUFS2 gene of patients with cardiomyopathy and encephalomyopathy Smeitink, J, Sengers, R, Smeets, R, van den Heuvel, L, Stöckler-Ipsiroglu, S, Mandel, H, Elpeleg, O, Trijbels, F, Loeffen, J	Ann. Neurol.	2001
24191001	Assembly factors for the membrane arm of human complex I Fearnley, IM, Carroll, J, Andrews, B, Walker, JE, Ding, S	Proc. Natl. Acad. Sci. U.S.A.	2013