The cell biology of polycystic kidney disease

被引：209

作者：

Chapin, Hannah C. ^{[1
]}

Caplan, Michael J. ^{[1
]}

机构：

[1] Yale Univ, Sch Med, Dept Cellular & Mol Physiol, New Haven, CT 06520 USA

来源：

JOURNAL OF CELL BIOLOGY | 2010年 / 191卷 / 04期

基金：

美国国家卫生研究院;

关键词：

DEPENDENT CHLORIDE SECRETION; PKD1; GENE-PRODUCT; N-TERMINAL KINASE; CYST FORMATION; C-JUN; EPITHELIAL-CELLS; CHANNEL ACTIVITY; MOLECULAR-BASIS; G-PROTEINS; E-CADHERIN;

D O I：

10.1083/jcb.201006173

中图分类号：

Q2 [细胞生物学];

学科分类号：

071013 [干细胞生物学];

摘要：

Polycystic kidney disease is a common genetic disorder in which fluid-filled cysts displace normal renal tubules Here we focus on autosomal dominant polycystic kidney disease, which is attributable to mutations in the PKD1 and PKD2 genes and which is characterized by perturbations of renal epithelial cell growth control, fluid transport, and morphogenesis The mechanisms that connect the under lying genetic defects to disease pathogenesis are poorly understood, but their exploration is shedding new light on interesting cell biological processes and suggesting novel therapeutic targets

引用

页码：701 / 710

页数：10

共 133 条

[1]

KCa3.1 potassium channels are critical for cAMP-dependent chloride secretion and cyst growth in autosomal-dominant polycystic kidney disease [J].

Albaqumi, Mamdouh ;

Srivastava, Shekhar ;

Li, Zhai ;

Zhdnova, Olga ;

Wulff, Heike ;

Itani, Omar ;

Wallace, Darren P. ;

Skolnik, Edward Y. .

KIDNEY INTERNATIONAL, 2008, 74 (06) :740-749

[2]

Regulation of ryanodine receptor-dependent calcium signaling by polycystin-2 [J].

Anyatonwu, Georgia I. ;

Estrada, Manuel ;

Tian, Xin ;

Somlo, Stefan ;

Ehrlich, Barbara E. .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2007, 104 (15) :6454-6459

[3]

The polycystic kidney disease 1 gene product mediates protein kinase C α-dependent and c-Jun N-terminal kinase-dependent activation of the transcription factor AP-1 [J].

Arnould, T ;

Kim, E ;

Tsiokas, L ;

Jochimsen, F ;

Grüning, W ;

Chang, JD ;

Walz, G .

JOURNAL OF BIOLOGICAL CHEMISTRY, 1998, 273 (11) :6013-6018

[4]

The N-terminal extracellular domain is required for polycystin-1-dependent channel activity [J].

Babich, V ;

Zeng, WZ ;

Yeh, BI ;

Ibraghimov-Beskrovanaya, O ;

Cai, YQ ;

Somlo, S ;

Huang, CL .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2004, 279 (24) :25582-25589

[5]

The ciliopathies: An emerging class of human genetic disorders [J].

Badano, Jose L. ;

Mitsuma, Norimasa ;

Beales, Phil L. ;

Katsanis, Nicholas .

ANNUAL REVIEW OF GENOMICS AND HUMAN GENETICS, 2006, 7 :125-148

[6]

Pkd1 Haploinsufficiency Increases Renal Damage and Induces Microcyst Formation following Ischemia/Reperfusion [J].

Bastos, Ana P. ;

Piontek, Klaus ;

Silva, Ana M. ;

Martini, Dino ;

Menezes, Luis F. ;

Fonseca, Jonathan M. ;

Fonseca, Ivone I. ;

Germino, Gregory G. ;

Onuchic, Luiz F. .

JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2009, 20 (11) :2389-2402

[7]

Polycystin-1 C-terminal Cleavage Is Modulated by Polycystin-2 Expression [J].

Bertuccio, Claudia A. ;

Chapin, Hannah C. ;

Cai, Yiqiang ;

Mistry, Kavita ;

Chauvet, Veronique ;

Somlo, Stefan ;

Caplan, Michael J. .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2009, 284 (31) :21011-21026

[8]

BESKROVNAYA OI, 2000, HUM MOL GENET, V9, P1641, DOI DOI 10.1093/HMG/9.11.1641

[9]

BESKROVNAYA OI, 1997, P NATL ACAD SCI USA, V94, P6397, DOI DOI 10.1073/PNAS.94.12.6397

[10]

PKD1 induces p21waf1 and regulation of the cell cycle via direct activation of the JAK-STAT signaling pathway in a process requiring PKD2 [J].

Bhunia, AK ;

Piontek, K ;

Boletta, A ;

Liu, LJ ;

Qian, F ;

Xu, PN ;

Germino, FJ ;

Germino, GG .

CELL, 2002, 109 (02) :157-168

← 1 2 3 4 5 6 7 8 9 10 →