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Cytosolic iron-sulfur cluster assembly factor NUBP1 (NUBP1)

Target: NUBP1
Molecular classification: Iron-sulfur (Fe-S) cluster assembly factor, P-loop NTPase, ATP-binding protein, Member of the Mrp/NBP35 ATP-binding proteins family, Component of cytosolic iron-sulfur protein assembly (CIA) machinery[2][4][6][7]

Overview

NUBP1 is a cytosolic iron-sulfur (Fe-S) cluster assembly factor that forms a heterotetrameric complex with NUBP2, functioning as a scaffold for the de novo assembly and transfer of Fe-S clusters to target apoproteins. It is a member of the Mrp/NBP35 family of ATP-binding proteins and is essential for the maturation of extramitochondrial Fe-S proteins. NUBP1 is implicated in pathways related to mitochondrial iron-sulfur cluster biogenesis and metabolic processes involving Fe-S proteins. Additionally, it plays a role in regulating centrosome duplication and cilium structure, though it is not currently considered a direct therapeutic target. Defects in NUBP1 function are associated with rare genetic diseases and broader cellular dysfunction due to impaired Fe-S protein maturation[2][4][6][7].

Other names: Nucleotide-binding protein 1 (NBP1)Nucleotide binding protein (E. coli MinD-like)NBPNBP 1NBP35Cytosolic Fe-S cluster assembly factor NUBP1CIAO5

Mechanism of action

null

Biological functions

Assembly of cytosolic iron-sulfur (Fe-S) clustersMaturation of extramitochondrial Fe-S proteinsFormation of Fe-S scaffold complex (in heterotetramer with NUBP2) for de novo Fe-S cluster biosynthesis and transfer to target apoproteinsRegulation of centrosome duplication (by similarity)Negative regulation of cilium formation and structure (by similarity)[2][6][4]

Disease associations

Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant 6Pica disease[2]Other potential roles relating to defects in iron-sulfur cluster protein assembly (based on pathway significance)[2]

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