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Fig. 8. BBS6 is enriched in organs affected in BBS and in ciliated cells. (A) Wholemount RNA in situ hybridization of an E10 mouse embryo showing strong BBS6 expression in the forebrain (Fb), retina (Re), branchial arches (Ba), developing heart (He) and limb bud (Lb). (B) Immunohistochemistry using BBS6 antibody; saggital section of a mouse E13.5 brain showing protein staining in the developing nervous system, including the neopallial cortex (Nc), the roof of the midbrain (Rm), the cerebellar primordium (Ce), the choroid plexus (Cp), the hypothalamus (Hy) and Rathke's pouch (Rp). Modest BBS6 protein staining is also detectable in the thalamus (Th) and the medulla oblongata (Mo). (C) In the adult kidney, BBS6 localizes to the ciliated border of renal tubular cells (Rtc). (D) In the adult photoreceptor, BBS6 is detected in the connecting cilium (Cc) and the inner and outer nuclear layers (Inl, Onl) but not the outer or inner segment (Os, Is). (E,F) BBS6 localizes to the ciliated layer of the olfactory epithelium (cl) as well as the ependymal layer (El) of ventricles (Ve); Lu, lumen. (G) A section of mouse testis is stained for BBS6 (green) and the PCM tube (antibody M4491; red). The area shown is taken from the periphery of a semineferous tubule containing both meiotic and spermatogenic cells. BBS6 colocalizes to centrosomes/basal bodies, but not the flagellar axoneme, in spermatogenic cells (see arrowheads). Also shown are nuclei (DAPI stained, blue) and a merged image.

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