Pediatric Nephrology

, Volume 32, Issue 4, pp 565–576 | Cite as

Vesicoureteral reflux and the extracellular matrix connection

  • Fatima Tokhmafshan
  • Patrick D. Brophy
  • Rasheed A. Gbadegesin
  • Indra R. GuptaEmail author


Primary vesicoureteral reflux (VUR) is a common pediatric condition due to a developmental defect in the ureterovesical junction. The prevalence of VUR among individuals with connective tissue disorders, as well as the importance of the ureter and bladder wall musculature for the anti-reflux mechanism, suggest that defects in the extracellular matrix (ECM) within the ureterovesical junction may result in VUR. This review will discuss the function of the smooth muscle and its supporting ECM microenvironment with respect to VUR, and explore the association of VUR with mutations in ECM-related genes.


Vesicoureteral reflux Ureterovesical junction Collagen Elastin Marfan syndrome Williams syndrome Cutis laxa Ehlers–Danlos syndrome Joint hypermobility Tenascin-XB 


Compliance with ethical standards

Conflict of interest

The authors declare no conflicts of interest.


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Copyright information

© IPNA 2016

Authors and Affiliations

  • Fatima Tokhmafshan
    • 1
  • Patrick D. Brophy
    • 2
  • Rasheed A. Gbadegesin
    • 3
    • 4
  • Indra R. Gupta
    • 1
    • 5
    Email author
  1. 1.Department of Human GeneticsMcGill UniversityMontrealCanada
  2. 2.Department of PediatricsUniversity of Iowa, Carver College of MedicineIowa CityUSA
  3. 3.Department of Pediatrics, Division of NephrologyDuke University Medical CenterDurhamUSA
  4. 4.Center for Human GeneticsDuke University Medical CenterDurhamUSA
  5. 5.Department of PediatricsMcGill UniversityMontrealCanada

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