A short carboxy-terminal domain of polycystin-1 reorganizes the microtubular network and the endoplasmic reticulum.
Mutations of PKD1 cause autosomal dominant polycystic kidney disease (ADPKD), a syndrome characterized by kidney cysts and progressive renal failure. Polycystin-1, the protein encoded by PKD1, is a large integral membrane protein with a short carboxy-terminal cytoplasmic domain that appears to initiate multiple cellular programs. We report now that this polycystin-1 domain contains a novel motif responsible for rearrangements of intermediate filaments, microtubules and the endoplasmic reticulum (ER). This motif reveals homology to CLIMP-63, a microtubule-binding protein that rearranges the ER. Our findings suggest that polycystin-1 influences the shape and localization of both the microtubular network and the ER.
Gao
Hongyu
H
Renal Division, University Hospital Freiburg, Freiburg, Germany.
Sellin
Lorenz K
LK
Pütz
Michael
M
Nickel
Christian
C
Imgrund
Michael
M
Gerke
Peter
P
Nitschke
Roland
R
Walz
Gerd
G
Kramer-Zucker
Albrecht G
AG