Pediatric Nephrology

, Volume 25, Issue 10, pp 2049–2059

Glomerulocystic kidney disease

Educational Review

DOI: 10.1007/s00467-009-1416-2

Cite this article as:
Bissler, J.J., Siroky, B.J. & Yin, H. Pediatr Nephrol (2010) 25: 2049. doi:10.1007/s00467-009-1416-2

Abstract

Glomerulocystic disease is a rare renal cystic disease with a long descriptive history. Findings from recent studies have significantly advanced the pathophysiological understanding of the disease processes leading to this peculiar phenotype. Many genetic syndromes associated with glomerulocystic disease have had their respective proteins localized to primary cilia or centrosomes. Transcriptional control of renal developmental pathways is dysregulated in obstructive diseases that also lead to glomerulocystic disease, emphasizing the importance of transcriptional choreography between renal development and renal cystic disease.

Keywords

Basal body Cilia Glomerulocystic kidney disease Polycystic kidney disease Tuberous sclerosis complex 

Copyright information

© IPNA 2010

Authors and Affiliations

  1. 1.Division of Nephrology and HypertensionCincinnati Children’s Hospital Medical CenterCincinnatiUSA
  2. 2.Division of PathologyCincinnati Children’s Hospital Medical CenterCincinnatiUSA

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