Reactome | NUBPL transfers 4Fe-4S to NDUFV1, V2

NUBPL transfers 4Fe-4S to NDUFV1, V2

Stable Identifier

R-HSA-6788556

Type

Reaction [uncertain]

Species

Homo sapiens

Compartment

mitochondrial matrix, mitochondrial inner membrane

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

In total, eight iron-sulfur (4Fe-4S) clusters are incorporated into six subunits (mitochondrial matrix-located NDUFS1, S2, S7, S8 and mitochondrial membrane-located V1 and V2) (Andrews et al. 2013). Incorporation into NDUFS1, S2, S7 and S8 is shown in a separate reaction. The process requires the presence of the co-chaperone complex HSPA9:HSCB (Maio et al., 2017). The mechanism of transfer is unknown. NDUFV1 also binds FMN (Schuelke et al. 1998).

Literature References

PubMed ID	Title	Journal	Year
9571201	Cloning of the human mitochondrial 51 kDa subunit (NDUFV1) reveals a 100% antisense homology of its 3'UTR with the 5'UTR of the gamma-interferon inducible protein (IP-30) precursor: is this a link between mitochondrial myopathy and inflammation? Schuelke, M, Loeffen, J, Mariman, E, Smeitink, J, van den Heuvel, L	Biochem. Biophys. Res. Commun.	1998
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