The role of properdin in complement-mediated renal diseases: a new player in complement-inhibiting therapy?

AbstractProperdin is known as the only positive regulator of the complement system. Properdin promotes the activity of this defense system by stabilizing its key enzymatic complexes: the complement alternative pathway (AP) convertases. Besides, some studies have indicated a role for properdin as an initiator of complement activity. Though the AP is a powerful activation route of the complement system, it is also involved in a wide variety of autoimmune and inflammatory diseases, many of which affect the kidneys. The role of properdin in regulating complement in health and disease has not received as much appraisal as the many negative AP regulators, such as factor H. Historically, properdin deficiency has been strongly associated with an increased risk for meningococcal disease. Yet only recently had studies begun to link properdin to other complement-related diseases, including renal diseases. In the light of the upcoming complement-inhibiting therapies, it is interesting whether properdin can be a therapeutic target to attenuate AP-mediated injury. A full understanding of the basic concepts of properdin biology is therefore needed. Here, we first provide an overview of the function of properdin in health and disease. Then, we explore its potential as a therapeutic target for the AP-associated renal diseases C3 glomerulopathy, atypical hemolytic uremic syndrome, and proteinuria-induced tubulointerstitial injury. Considering current knowledge, properdin-inhibiting therapy see...
Source: Pediatric Nephrology - Category: Urology & Nephrology Source Type: research

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Source: Acta Neurologica Belgica - Category: Neurology Source Type: research
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Source: Blood Research - Category: Hematology Tags: Blood Res Source Type: research
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Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
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Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
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Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
Conditions:   Vasculitis;   Systemic Lupus Erythematosus;   Myositis Intervention:   Other: ICU Care Sponsors:   University of Göttingen;   University Medical Center Goettingen Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Authors: Nozue T, Toyoshima M, Fukada A, Suda T Abstract A 71-year-old woman with congenital rubella syndrome (CRS) presented with prolonged cough. No physical findings suggested the presence of any connective tissue diseases. Chest computed tomography showed ground-glass opacities and consolidations in the bilateral lower lobes. She had elevated serum Krebs von den Lungen-6, hypoxemia and positive serum anti-Jo-1 antibody. Bronchoalveolar lavage fluid revealed lymphocytosis with a decreased CD4/CD8 ratio. A transbronchial lung biopsy specimen revealed organizing pneumonia. Based on a diagnosis of interstitial pneu...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
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Source: Drugs.com - Pharma News - Category: Pharmaceuticals Source Type: news
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Source: Clinical and Experimental Nephrology - Category: Urology & Nephrology Source Type: research
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Source: Journal of the Association of Physicians of India - Category: General Medicine Tags: J Assoc Physicians India Source Type: research
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