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Luo Y
,
Vassilev PM
,
Li X
,
Kawanabe Y
,
Zhou J
.
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Mutations in polycystin 2 (PC2), a Ca(2+)-permeable cation channel, cause autosomal dominant polycystic kidney disease. Whether PC2 functions in the endoplasmic reticulum (ER) or in the plasma membrane has been controversial. Here we generated and characterized a polyclonal antibody against PC2, determined the subcellular localization of both endogenous and transfected PC2 by immunohistochemistry and biotinylation of cell surface proteins, and assessed PC2 channel properties with electrophysiology. Endogenous PC2 was found in the plasma membrane and the primary cilium of mouse inner medullar collecting duct (IMCD) cells and Madin-Darby canine kidney (MDCK) cells, whereas heterologously expressed PC2 showed a predominant ER localization. Patch-clamping of IMCD cells expressing endogenous or heterologous PC2 confirmed the presence of the channel on the plasma membrane. Treatment with chaperone-like factors facilitated the translocation of the PC2 channel to the plasma membrane from intracellular pools. The unitary conductances, channel kinetics, and other characteristics of both endogenously and heterologously expressed PC2 were similar to those described in our previous study in Xenopus laevis oocytes. These results show that PC2 functions as a plasma membrane channel in renal epithelia and suggest that PC2 contributes to Ca(2+) entry and transport of other cations in defined nephron segments in vivo.
Brown,
Correcting temperature-sensitive protein folding defects.
1997, Pubmed
Brown,
Correcting temperature-sensitive protein folding defects.
1997,
Pubmed
Cai,
Identification and characterization of polycystin-2, the PKD2 gene product.
1999,
Pubmed
Chen,
Polycystin-L is a calcium-regulated cation channel permeable to calcium ions.
1999,
Pubmed
,
Xenbase
Delmas,
Constitutive activation of G-proteins by polycystin-1 is antagonized by polycystin-2.
2002,
Pubmed
Denning,
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive.
1992,
Pubmed
,
Xenbase
Foggensteiner,
Cellular and subcellular distribution of polycystin-2, the protein product of the PKD2 gene.
2000,
Pubmed
Geng,
Identification and localization of polycystin, the PKD1 gene product.
1996,
Pubmed
Gonzalez-Perrett,
Voltage dependence and pH regulation of human polycystin-2-mediated cation channel activity.
2002,
Pubmed
González-Perrett,
Polycystin-2, the protein mutated in autosomal dominant polycystic kidney disease (ADPKD), is a Ca2+-permeable nonselective cation channel.
2001,
Pubmed
Guo,
Identification and characterization of a novel polycystin family member, polycystin-L2, in mouse and human: sequence, expression, alternative splicing, and chromosomal localization.
2000,
Pubmed
Hamill,
Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches.
1981,
Pubmed
Hanaoka,
Co-assembly of polycystin-1 and -2 produces unique cation-permeable currents.
,
Pubmed
Hou,
Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease.
2002,
Pubmed
Hughes,
The polycystic kidney disease 1 (PKD1) gene encodes a novel protein with multiple cell recognition domains.
1995,
Pubmed
Hughes,
Identification of a human homologue of the sea urchin receptor for egg jelly: a polycystic kidney disease-like protein.
1999,
Pubmed
Ibraghimov-Beskrovnaya,
Polycystin: in vitro synthesis, in vivo tissue expression, and subcellular localization identifies a large membrane-associated protein.
1997,
Pubmed
Jensen,
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.
1995,
Pubmed
Koulen,
Polycystin-2 is an intracellular calcium release channel.
2002,
Pubmed
Mochizuki,
PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein.
1996,
Pubmed
NULL,
Polycystic kidney disease: the complete structure of the PKD1 gene and its protein. The International Polycystic Kidney Disease Consortium.
1995,
Pubmed
Nauli,
Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells.
2003,
Pubmed
Newby,
Identification, characterization, and localization of a novel kidney polycystin-1-polycystin-2 complex.
2002,
Pubmed
Nomura,
Identification of PKDL, a novel polycystic kidney disease 2-like gene whose murine homologue is deleted in mice with kidney and retinal defects.
1998,
Pubmed
Parnell,
Polycystin-1 activation of c-Jun N-terminal kinase and AP-1 is mediated by heterotrimeric G proteins.
2002,
Pubmed
Pazour,
The intraflagellar transport protein, IFT88, is essential for vertebrate photoreceptor assembly and maintenance.
2002,
Pubmed
Pazour,
Polycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney disease.
2002,
Pubmed
Peters,
Cellular localization and tissue distribution of polycystin-1.
1999,
Pubmed
Qian,
PKD1 interacts with PKD2 through a probable coiled-coil domain.
1997,
Pubmed
Sato,
Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.
1996,
Pubmed
Scheffers,
Distinct subcellular expression of endogenous polycystin-2 in the plasma membrane and Golgi apparatus of MDCK cells.
2002,
Pubmed
Taulman,
Polaris, a protein involved in left-right axis patterning, localizes to basal bodies and cilia.
2001,
Pubmed
,
Xenbase
Tsien,
Measurement of cytosolic free Ca2+ with quin2.
1989,
Pubmed
Tsiokas,
Homo- and heterodimeric interactions between the gene products of PKD1 and PKD2.
1997,
Pubmed
Tsiokas,
Specific association of the gene product of PKD2 with the TRPC1 channel.
1999,
Pubmed
Valentijn,
Biosynthesis and processing of epithelial sodium channels in Xenopus oocytes.
1998,
Pubmed
,
Xenbase
Vassilev,
Polycystin-2 is a novel cation channel implicated in defective intracellular Ca(2+) homeostasis in polycystic kidney disease.
2001,
Pubmed
,
Xenbase
Ward,
Degradation of CFTR by the ubiquitin-proteasome pathway.
1995,
Pubmed
Wu,
Identification of PKD2L, a human PKD2-related gene: tissue-specific expression and mapping to chromosome 10q25.
1998,
Pubmed
Yoder,
Polaris, a protein disrupted in orpk mutant mice, is required for assembly of renal cilium.
2002,
Pubmed
Yuasa,
The sequence, expression, and chromosomal localization of a novel polycystic kidney disease 1-like gene, PKD1L1, in human.
2002,
Pubmed
