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Human Molecular Genetics Advance Access published online on January 20, 2005
Human Molecular Genetics, doi:10.1093/hmg/ddi061
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Human Molecular Genetics © Oxford University Press 2005; all rights reserved

Article

Characterization of the nephrocystin/nephrocystin-4 complex and subcellular localization of nephrocystin-4 to primary cilia and centrosomes

Géraldine Mollet 1, Flora Silbermann 1, Marion Delous 1, Rémi Salomon 2, Corinne Antignac 3*, and Sophie Saunier 1

1 Inserm U574, Hôpital Necker-Enfants Malades, Université René Descartes, Paris, France
2 Inserm U574, Hôpital Necker-Enfants Malades, Université René Descartes, Paris, France; Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants Malades, Université René Descartes, Paris, France
3 Inserm U574, Université René Descartes, Hôpital Necker-Enfants Malades, 149, rue de Sèvres, 75015 Paris, France; Service de Génétique, Hôpital Necker-Enfants Malades, Université René Descartes, Paris, France

* To whom correspondence should be addressed.
Corinne Antignac, E-mail: antignac{at}necker.fr


  Abstract

Nephrocystin and nephrocystin-4 are newly identified proteins involved in familial juvenile nephronophthisis, an autosomal recessive nephropathy characterized by cyst formation and renal fibrosis. Nephrocystin is an adaptor protein that is able to associate with signaling molecules involved in cell adhesion and actin cytoskeleton organization, such as p130Cas, Pyk2, tensin and filamins. Nephrocystin was recently shown to interact and to colocalize with the microtubule component {beta}-tubulin to the primary cilia in renal epithelial cells, an organelle known to play a key role in the pathogenesis of cystic kidney diseases. In this study, we demonstrated that nephrocystin-4 also localizes to the primary cilia in polarized epithelial tubular cells, particularly at the basal bodies, and associates with microtubule component {alpha}-tubulin, suggesting a common role for the nephrocystin proteins in ciliary function. However, the colocalization of nephrocystin-4 with the microtubules is not restricted to the primary cilia, as nephrocystin-4 was also detected at the centrosomes of dividing cells and close to the cortical actin cytoskeleton in polarized cells. We also detected p130Cas and Pyk2 in the nephrocystin-4-containing complex, confirming the role of the nephrocystin proteins in cell-cell and cell-matrix adhesion signaling events. Finally, we refined the structural and functional regions involved in the interaction between nephrocystin and nephrocystin-4. These data suggest that nephrocystin and nephrocystin-4 belong to a multifunctional complex localized in actin- and microtubule-based structures involved in cell-cell and cell-matrix adhesion signaling as well as in cell division.


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