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Phenotypes

Mouse phenotype:

abnormal retina morphology, cataract, abnormal vitreous body morphology, abnormal lens morphology

Subcellular localization

Ciliary associated gene, Centrosome, Cytosol

Functional category

• Motors (dynein/kinesin)
• Ciliary assembly/disassembly

Function

Smo activates the Gαi-LGN-NuMA-dynein axis, causing accumulation of a portion of OFD1 at centrioles in early ciliogenesis. Both pathways are critical for redistribution of BBS4 from satellites to centrioles, which is also mediated by OFD1 centriolar translocation. Notably, different Smo agonists, which activate Smo distinctly, activate one or the other of these pathways, only in combination they recapitulate the activity of Hh ligand. These studies provide new insight into physiological stimuli (Hh) that activate autophagy and promote ciliogenesis and introduce a novel role for the Gαi-LGN-NuMA-dynein complex in this process(33258871).