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Related Experiment Video

Updated: Jun 17, 2026

Mouse Model of Alloimmune-induced Vascular Rejection and Transplant Arteriosclerosis
07:05

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Interstitial inflammation in Alport syndrome.

Jan Jedlicka1, Afschin Soleiman, Dan Draganovici

  • 1Medizinische Poliklinik Campus Innenstadt, University of Munich, 80336 Munich, Germany.

Human Pathology
|December 17, 2009
PubMed
Summary

Interstitial T cell infiltration, marked by CXCR3, is prominent in Alport syndrome kidneys and correlates with disease severity. Targeting these T cells may offer a new treatment strategy for Alport syndrome progression.

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Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • Alport syndrome is a hereditary kidney disease caused by collagen IV defects.
  • Interstitial lymphocyte infiltration is implicated in Alport syndrome progression in mouse models.
  • CXCR3 is a chemokine receptor crucial for lymphocyte homing to the kidneys.

Purpose of the Study:

  • To investigate the role of CXCR3-positive T cells in human Alport syndrome.
  • To correlate interstitial CXCR3-positive cell infiltration with disease severity and renal function.

Main Methods:

  • Immunohistochemistry on kidney biopsies from Alport syndrome patients, IgA nephropathy patients, and healthy donors.
  • Analysis of collagen IV alpha5 chain, CXCR3, and CD3-positive T cells.
  • Correlation analysis between cell infiltration and clinical/morphological parameters.

Main Results:

  • Alport syndrome biopsies showed loss of collagen IV alpha5 and interstitial accumulation of CXCR3-positive cells.
  • Most CXCR3-positive cells were CD3-positive T cells.
  • Interstitial CXCR3-positive cell counts correlated with serum creatinine and progressive morphologic features.

Conclusions:

  • Human Alport syndrome is associated with interstitial CD3 and CXCR3 T-cell infiltration.
  • The extent of this infiltration correlates with impaired renal function and disease progression.
  • Targeting CXCR3-positive T cells could be a potential therapeutic strategy for Alport syndrome.