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OCIA domain-containing protein 2 (OCIAD2) is an inner mitochondrial membrane protein that serves as an assembly factor for the dimeric complex III (CIII₂) of the mitochondrial electron transport chain. Loss of OCIAD2 impairs the proper assembly and enzymatic activity of complex III, leading to abnormal mitochondrial morphology and diminished complex III activity, while not affecting complexes I or IV. OCIAD2 interacts with electron transport chain proteins and stabilizes CIII₂-containing supercomplexes. It also participates in signaling pathways by activating STAT3 and is implicated in cell migration, which is significant in tumor metastasis. Dysregulation of OCIAD2 expression has been observed in several cancers and neurodegenerative conditions, highlighting its importance in cellular metabolism and disease biology.
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