NUBPL binds 4Fe-4S

Stable Identifier
R-HSA-5690023
Type
Reaction [binding]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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The iron-sulfur protein NUBPL is thought to bind the cofactor [4Fe-4S] cluster and deliver it to complex I (NADH dehydrogenase) subunits during its biogenesis. The exact mechanism of transfer is unknown but defects in NUBPL are shown to cause mitochondrial complex I deficiency (MT-C1D) with a distinct MRI pattern (Sheftel et al. 2009, Kevelam et al. 2013).
Literature References
PubMed ID Title Journal Year
23553477 NUBPL mutations in patients with complex I deficiency and a distinct MRI pattern

Lunsing, RJ, Rating, D, Rodenburg, RJ, Kevelam, SH, Ferreira, P, Mitchell, A, Abbink, TE, Arroyo, HA, Nijtmans, LG, Vanderver, A, Wolf, NI, Heutink, P, van der Knaap, MS, van Berkel, CG

Neurology 2013
19752196 Human ind1, an iron-sulfur cluster assembly factor for respiratory complex I

Kerscher, S, Netz, DJ, Pierik, AJ, Lill, R, Wittig, I, Elsässer, HP, Balk, J, Stehling, O, Sheftel, AD, Brandt, U

Mol. Cell. Biol. 2009
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