Abstract
It has recently been reported (McEwen, et al PNAS 2007) that nephrocystin 6 (NPHP6) localizes to the olfactory epithelium (OE), and that mutations in NPHP6 can cause anosmia in addition to renal cystic disease. We demonstrate here that other proteins known to cause renal cystic disease ‐ polycystin 1 (PKD1) and meckelin (MKS3) ‐ also localize to the murine OE. MKS3 and PKD1 are both detected in the OE by RT‐PCR (products were sequenced to confirm their identity). Using previously characterized antibodies, we demonstrated that MKS3 localizes specifically to the dendritic knobs of mature olfactory neurons, whereas PKD1 localizes the cell bodies and cilia of mature olfactory neurons. The expression of MKS3 is developmentally regulated, with little or no expression detected postnatally until P7, and the strongest expression detected in adult mice. In addition, in mice null for PKD1 in the OE, MKS3 expression is reduced. In conclusion, proteins associated with renal cystic disease are expressed in the OE where they localize to the knobs and cilia of mature olfactory neurons.
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