| Interstitial inflammation in Alport syndrome. | |
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MedLine Citation:
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PMID: 20004949 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The Alport syndrome is a hereditary glomerular disease linked to structural abnormalities of collagen IV. In a mouse model of Alport syndrome, the interstitial lymphocyte influx was important for disease progression. CXCR3 is a chemokine receptor involved in lymphocyte recruitment to the kidney. We hypothesized that CXCR3-positive T cells might be involved in human Alport syndrome. Immunohistochemistry was performed on formalin-fixed, paraffin-embedded biopsies from 17 patients with Alport syndrome, 10 with immunoglobulin A (IgA) nephropathy, and 11 healthy donor kidneys. We investigated the expression of the alpha5 chain of collagen IV to confirm the morphologic diagnosis, the chemokine receptor CXCR3 and CD3-positive T cells. Alport syndrome biopsies demonstrated a complete loss of the alpha5 chain of collagen IV from the glomerular basement membrane and the morphologic features consistent with Alport syndrome on electron microscopy. A prominent number of CXCR3-positive cells were found in the tubulointerstitium. Most of the CXCR3-positive cells were CD3-positive T cells, demonstrated by double-labeling in selected biopsies. The number of CXCR3-positive cells in kidneys with Alport syndrome correlated with serum creatinine (P < .05) and with morphologic features of a progressive disease (eg, interstitial fibrosis, glomerulosclerosis, and tubular atrophy). The severity of interstitial CXCR3-positive cell influx was similar in Alport syndrome as compared to immunoglobulin A nephropathy. The noninflammatory glomerular lesion of Alport syndrome is associated with prominent interstitial accumulation of CD3- and CXCR3-positive lymphocytes. The degree of infiltration correlated with renal function. We speculate that targeting T lymphocytes, for example, by CXCR3 blocking agents, might be a novel approach to inhibit disease progression in patients with Alport syndrome. |
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Authors:
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Jan Jedlicka; Afschin Soleiman; Dan Draganovici; Jana Mandelbaum; Urs Ziegler; Heinz Regele; Rudolf P W?thrich; Oliver Gross; Hans-Joachim Anders; Stephan Segerer |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2009-12-11 |
Journal Detail:
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Title: Human pathology Volume: 41 ISSN: 1532-8392 ISO Abbreviation: Hum. Pathol. Publication Date: 2010 Apr |
Date Detail:
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Created Date: 2010-03-15 Completed Date: 2010-03-31 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9421547 Medline TA: Hum Pathol Country: United States |
Other Details:
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Languages: eng Pagination: 582-93 Citation Subset: IM |
Copyright Information:
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Copyright 2010 Elsevier Inc. |
Affiliation:
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Medizinische Poliklinik Campus Innenstadt, University of Munich, 80336 Munich, Germany. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Animals Antigens, CD3 / metabolism Child Child, Preschool Female Humans Male Nephritis, Hereditary / immunology*, pathology Nephritis, Interstitial / immunology*, pathology Receptors, CXCR3 / metabolism T-Lymphocytes / immunology, metabolism Young Adult |
| Chemical | |
Reg. No./Substance:
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0/Antigens, CD3; 0/CXCR3 protein, human; 0/Receptors, CXCR3 |
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