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Letter

Nature Genetics 36, 994–998 (1 September 2004) | doi:10.1038/ng1418

Loss of BBS proteins causes anosmia in humans and defects in olfactory cilia structure and function in the mouse

Heather M Kulaga , Carmen C Leitch , Erica R Eichers , Jose L Badano , Alysa Lesemann , Bethan E Hoskins , James R Lupski , Philip L Beales , Randall R Reed & Nicholas Katsanis

Defects in cilia are associated with several human disorders, including Kartagener syndrome, polycystic kidney disease, nephronophthisis and hydrocephalus. We proposed that the pleiotropic phenotype of Bardet-Biedl syndrome (BBS), which encompasses retinal degeneration, truncal obesity, renal and limb malformations and developmental delay, is due to dysfunction of basal bodies and cilia.