Cytosolic iron-sulfur assembly component 1 (CIAO1)

Cytosolic iron-sulfur assembly component 1 (CIAO1) is a WD40-repeat containing protein that is a core component of the cytosolic iron-sulfur protein assembly (CIA) complex. It mediates the incorporation of iron-sulfur clusters into a wide range of extramitochondrial Fe-S proteins involved in essential cellular activities such as metabolic pathways and DNA replication/repair. CIAO1 forms part of a multi-protein targeting complex with MMS19 and CIAO2A/CIAO2B, facilitating the last step of Fe-S cluster transfer to target proteins in the cytoplasm and nucleus. Dysfunction of CIAO1 is associated with severe mitochondrial and neurodegenerative syndromes due to the vital roles of its protein products and their cofactors in cell homeostasis. While CIAO1 is not yet a direct drug development target, WD-repeat proteins as a family are being investigated for their therapeutic potential in cancer, metabolism, neurology, and chromatin modification diseases.

Other names

CIAO1CIA1WDR39WD repeat domain 39WD repeat-containing protein 39WD40 protein Ciao1MMDS10cytosolic iron-sulfur assembly 1 homologprobable cytosolic iron-sulfur protein assembly protein CIAO1

No direct drugs; not considered a classic druggable target, mechanisms relate to modulation of protein-protein interactions in multi-protein iron-sulfur complex if targeted