Human pathology

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BBS8

MIM.608132 14q32.1

TTC8

BBS8 is a tetratricopeptide repeat protein that contains 8 TPR domains (TTC8) and shows similarity to a prokaryotic domain pilF that is involved in pilus formation and twitching mobility.

Functions

- BBS8, as BBS4, localizes to centrosomes and basal bodies.

- BBS8 shows limited similarity to the budding yeast cdc23 protein, a member of the APC, although the biological relevance of these findings needs further evaluation.

Pathology

- BBS8 is mutated in some families with Bardet-Biedl syndrome (MIM.209900).