The pathogenesis of polycystic kidney disease

F. A. Carone, Robert Bacallao, Y. S. Kanwar

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Polycystic kidney disease (PKD) is a genetic or acquired disorder characterized by progressive distention of multiple tubular segments and manifested by fluid accumulation, growth of non-neoplastic epithelial cells and remodeling of the extracellular matrix resulting ultimately in some degree of renal functional impairment, with the potential for regression following removal of the inductive agent(s). It is due to an aberration of one or more factors regulating tubular morphogenesis. Human PKD can pursue a rapid course with renal failure occurring perinatally (infantile PKD) or an indolent course without renal failure developing during the life of the individual (adult PKD). Human acquired PKD develops in atrophic and scarred end-stage kidneys with non-cystic forms of renal disease. Cell proliferation, fluid secretion, impaired cell-cell and cell-matrix interaction, defective function of the Golgi apparatus, cell undifferentiation, and an abnormal matrix have been implicated in the pathogenesis of PKD based on clinical and experimental studies. Under normal conditions, the dynamic turnover of tubular epithelia and matrices are tightly regulated to maintain tubular morphology. The basic defect in PKD is tubular dysmorphogenesis. Our finding indicates that the principal phenotypic features of autosomal dominant PKD (ADPKD) are altered structure and function of the Golgi complex, altered structure and composition of the matrix and cell undifferentiation, all of which are probably interrelated. If the gene product of the ADPKD 1 gene results in a defective matrix, the abnormal Golgi function and cell differentiation may be due to faulty matrix-cell communication.

Original languageEnglish (US)
Pages (from-to)213-221
Number of pages9
JournalHistology and Histopathology
Volume10
Issue number1
StatePublished - 1995
Externally publishedYes

Fingerprint

Polycystic Kidney Diseases
Autosomal Dominant Polycystic Kidney
Golgi Apparatus
Kidney
Cell Communication
Renal Insufficiency
Fluids and Secretions
Morphogenesis
Genes
Extracellular Matrix
Cell Differentiation
Epithelium
Epithelial Cells
Cell Proliferation
Growth

Keywords

  • Cell proliferation
  • Cell-Matrix interaction
  • Fluid secretion
  • Polycystic kidney disease

ASJC Scopus subject areas

  • Cell Biology
  • Anatomy
  • Histology
  • Pathology and Forensic Medicine

Cite this

Carone, F. A., Bacallao, R., & Kanwar, Y. S. (1995). The pathogenesis of polycystic kidney disease. Histology and Histopathology, 10(1), 213-221.

The pathogenesis of polycystic kidney disease. / Carone, F. A.; Bacallao, Robert; Kanwar, Y. S.

In: Histology and Histopathology, Vol. 10, No. 1, 1995, p. 213-221.

Research output: Contribution to journalArticle

Carone, FA, Bacallao, R & Kanwar, YS 1995, 'The pathogenesis of polycystic kidney disease', Histology and Histopathology, vol. 10, no. 1, pp. 213-221.
Carone, F. A. ; Bacallao, Robert ; Kanwar, Y. S. / The pathogenesis of polycystic kidney disease. In: Histology and Histopathology. 1995 ; Vol. 10, No. 1. pp. 213-221.
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