A mechanistic approach to inherited polycystic kidney disease

John Bissler, Bradley P. Dixon

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

There are approximately six and a half million people, of the estimated world population of six billion, with inherited polycystic kidney disease. Polycystic kidney diseases have a broad spectrum of associated findings that distinguish and define them as specific disease states. The dysregulation of renal tubular epithelial cell biology, including cell polarity, cell signaling, proliferation and apoptosis, basement membrane and matrix abnormalities, and fluid transport, has been postulated to contribute to cystogenesis. Evidence is currently accumulating that supports an association of the primary cilium and basal body, as well as the focal adhesion assembly, with polycystic kidney diseases. Renal cystogenesis may be the result of a disruption of a critical feedback loop that regulates tissue morphology based on the epithelial cell environment.

Original languageEnglish (US)
Pages (from-to)558-566
Number of pages9
JournalPediatric Nephrology
Volume20
Issue number5
DOIs
StatePublished - May 1 2005
Externally publishedYes

Fingerprint

Polycystic Kidney Diseases
Epithelial Cells
Basal Bodies
Kidney
Cell Polarity
Focal Adhesions
Cilia
Basement Membrane
Cell Biology
Cell Proliferation
Apoptosis
Population

All Science Journal Classification (ASJC) codes

  • Nephrology
  • Pediatrics, Perinatology, and Child Health

Cite this

A mechanistic approach to inherited polycystic kidney disease. / Bissler, John; Dixon, Bradley P.

In: Pediatric Nephrology, Vol. 20, No. 5, 01.05.2005, p. 558-566.

Research output: Contribution to journalArticle

Bissler, John ; Dixon, Bradley P. / A mechanistic approach to inherited polycystic kidney disease. In: Pediatric Nephrology. 2005 ; Vol. 20, No. 5. pp. 558-566.
@article{d1553c2d08824f8eb812d96d681f6405,
title = "A mechanistic approach to inherited polycystic kidney disease",
abstract = "There are approximately six and a half million people, of the estimated world population of six billion, with inherited polycystic kidney disease. Polycystic kidney diseases have a broad spectrum of associated findings that distinguish and define them as specific disease states. The dysregulation of renal tubular epithelial cell biology, including cell polarity, cell signaling, proliferation and apoptosis, basement membrane and matrix abnormalities, and fluid transport, has been postulated to contribute to cystogenesis. Evidence is currently accumulating that supports an association of the primary cilium and basal body, as well as the focal adhesion assembly, with polycystic kidney diseases. Renal cystogenesis may be the result of a disruption of a critical feedback loop that regulates tissue morphology based on the epithelial cell environment.",
author = "John Bissler and Dixon, {Bradley P.}",
year = "2005",
month = "5",
day = "1",
doi = "10.1007/s00467-004-1665-z",
language = "English (US)",
volume = "20",
pages = "558--566",
journal = "Pediatric Nephrology",
issn = "0931-041X",
publisher = "Springer Verlag",
number = "5",

}

TY - JOUR

T1 - A mechanistic approach to inherited polycystic kidney disease

AU - Bissler, John

AU - Dixon, Bradley P.

PY - 2005/5/1

Y1 - 2005/5/1

N2 - There are approximately six and a half million people, of the estimated world population of six billion, with inherited polycystic kidney disease. Polycystic kidney diseases have a broad spectrum of associated findings that distinguish and define them as specific disease states. The dysregulation of renal tubular epithelial cell biology, including cell polarity, cell signaling, proliferation and apoptosis, basement membrane and matrix abnormalities, and fluid transport, has been postulated to contribute to cystogenesis. Evidence is currently accumulating that supports an association of the primary cilium and basal body, as well as the focal adhesion assembly, with polycystic kidney diseases. Renal cystogenesis may be the result of a disruption of a critical feedback loop that regulates tissue morphology based on the epithelial cell environment.

AB - There are approximately six and a half million people, of the estimated world population of six billion, with inherited polycystic kidney disease. Polycystic kidney diseases have a broad spectrum of associated findings that distinguish and define them as specific disease states. The dysregulation of renal tubular epithelial cell biology, including cell polarity, cell signaling, proliferation and apoptosis, basement membrane and matrix abnormalities, and fluid transport, has been postulated to contribute to cystogenesis. Evidence is currently accumulating that supports an association of the primary cilium and basal body, as well as the focal adhesion assembly, with polycystic kidney diseases. Renal cystogenesis may be the result of a disruption of a critical feedback loop that regulates tissue morphology based on the epithelial cell environment.

UR - http://www.scopus.com/inward/record.url?scp=21244468364&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=21244468364&partnerID=8YFLogxK

U2 - 10.1007/s00467-004-1665-z

DO - 10.1007/s00467-004-1665-z

M3 - Article

VL - 20

SP - 558

EP - 566

JO - Pediatric Nephrology

JF - Pediatric Nephrology

SN - 0931-041X

IS - 5

ER -