Journal
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
Volume 15, Issue 3, Pages 592-602Publisher
AMER SOC NEPHROLOGY
DOI: 10.1097/01.ASN.0000113793.12558.1D
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- NIDDK NIH HHS [DK32520] Funding Source: Medline
- NIGMS NIH HHS [R01 GM030626] Funding Source: Medline
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Recent evidence suggests that structural and functional abnormalities of primary cilia in kidney epithelia are associated with mouse and human autosomal dominant polycystic kidney disease. To determine whether fibrocystin/polyductin/tigmin (FPC), the protein product encoded by the PKHDI gene that is responsible for autosomal recessive polycystic kidney disease among human subjects, is also a component of primary cilia in the kidney, antipeptide antibodies to the carboxyl-terminal intracellular domain and amino-terminal extracellular domain of FPC were generated and were characterized with immunoblotting, and immuno-light and -electron microscopy. Immunolocalization in normal kidney tissue sections and cultured kidney cells demonstrated that FPC was localized to the primary cilia and concentrated on the basal bodies in both kidney tissue sections and cultured kidney cells. The FPC expression pattern was not altered in kidney cells with PkdI mutations. These findings suggest that FPC is a functional and/or structural component of primary cilia in kidney tubular cells. It is proposed that the pathogenesis of autosomal recessive polycystic kidney disease is linked to the dysfunction of primary cilia.
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