USP8

Cilia effects upon perturbation of USP8

Cilia number / % ciliated:

Incrased cilia number

Loss-of-function effect:

Longer cilia

Ciliogenesis screen results (4 screens)

• Kim2016: No effect
• Wheway et al. 2015 (siRNA) [siRNA]: Ciliogenesis Defect (z=-2.56) PMID:26167766
• Breslow et al. 2018 (CRISPR) [CRISPR]: Positive Regulator (Hh signaling, casTLE effect=-2.73) PMID:29459680
• Roosing et al. 2015 (siRNA) [siRNA]: Hyper-ciliogenesis (robust z=2.86, ciliated=59.5%) PMID:26595381

Subcellular localization

basal body, cytosol, endosome, nucleus

Functional category

• Ciliary assembly/disassembly
• Trafficking (BBSome, small GTPases, vesicular transport, ATPases)
• Actin & cytoskeleton regulation

Function

Binds to, stabilises and deubiquiti tes HIF1伪 to control ciliogenesis, maintaining a basal expression of HIF1伪 (24378640). Recruited by SUMOylated Smo to antagonise Smo ubiquiti tion and degradation, leading to increased Hh pathway activity (27746045). USP8 phosphorylation by EGFR is essential for suppression of ciliogenesis, indicating that mutations or lack of USP8 function may cause ciliopathies through elongation of primary cilia (29472535).