NUBPL binds 4Fe-4S

Stable Identifier
Reaction [binding]
Homo sapiens
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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
19752196 Human ind1, an iron-sulfur cluster assembly factor for respiratory complex I

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

Mol. Cell. Biol. 2009
23553477 NUBPL mutations in patients with complex I deficiency and a distinct MRI pattern

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

Neurology 2013
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