Homo- and heterodimeric interactions between the gene products of PKD1 and PKD2

Leonidas Tsiokas, Emily Kim, Thierry Arnould, Vikas P. Sukhatme, Gerd Walz

Résultats de recherche: Contribution à un journal/une revueArticleRevue par des pairs

Résumé

PKD1 and PKD2 are two recently identified genes that are responsible for the vast majority of autosomal polycystic kidney disease, a common inherited disease that causes progressive renal failure. PKD1 encodes polycystin, a large glycoprotein that contains several extracellular motifs indicative of a role in cell-cell or cell-matrix interactions, and the PKD2 encodes a protein with homology to a voltage-activated calcium channel and to PKD1. It is currently unknown how mutations of either protein functionally cause autosomal polycystic kidney disease. We show that PKD1 and PKD2 interact through their C-terminal cytoplasmic tails. This interaction resulted in an up-regulation of PKD1 but not PKD2. Furthermore, the cytoplasmic tail of PKD2 but not PKD1 formed homodimers through a coiled-coil domain distinct from the region required for interaction with PKD1. These interactions suggest that PKD1 and PKD2 may function through a common signaling pathway that is necessary for normal tubulogenesis and that PKD1 may require the presence of PKD2 for stable expression.

langue originaleAnglais
Pages (de - à)6965-6970
Nombre de pages6
journalProceedings of the National Academy of Sciences of the United States of America
Volume94
Numéro de publication13
Les DOIs
Etat de la publicationPublié - 24 juin 1997

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