Complement receptor 3 mediates renal protection in experimental C3 glomerulopathy
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چکیده
منابع مشابه
Complement receptor 3 mediates renal protection in experimental C3 glomerulopathy
C3 glomerulopathy is a complement-mediated renal disease that is frequently associated with abnormalities in regulation of the complement alternative pathway. Mice with deficiency of factor H (Cfh(-/-)), a negative alternative pathway regulator, are an established experimental model of C3 glomerulopathy in which complement C3 fragments including iC3b accumulate along the glomerular basement mem...
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The complement system is an essential part of innate immunity acting as afirst-line defense against infection and provides an interface between innate and adaptive immunity (1,2). It consists of a network of soluble (fluid phase) and cell membrane proteins (solid phase). The key step in the complement cascade is the cleavage of C3 to C3a and C3b leading to the formation of a C3 convertase via t...
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Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are widely recognized subtypes of C3 glomerulopathy. These ultra-rare renal diseases are characterized by fluid-phase dysregulation of the alternative complement pathway that leads to deposition of complement proteins in the renal glomerulus. Disease triggers are unknown and because targeted treatments are lacking, progress to end sta...
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C3 glomerulopathy is a recently defined entity that encompasses a group of kidney diseases caused by abnormal control of complement activation with deposition of complement component C3 in glomeruli leading to variable glomerular inflammation. Before the recognition of the unique pathogenesis of these cases, they were variably classified according to their morphological features. C3 glomerulopa...
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Abnormal regulation of the complement alternative pathway is associated with C3 glomerulopathy. Complement factor H is the main plasma regulator of the alternative pathway and consists of 20 short consensus repeat (SCR) domains. Although recombinant full-length factor H represents a logical treatment for C3 glomerulopathy, its production has proved challenging. We and others have designed recom...
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ژورنال
عنوان ژورنال: Kidney International
سال: 2016
ISSN: 0085-2538
DOI: 10.1016/j.kint.2015.11.024