C3(H2O) prevents rescue of complement-mediated C3 glomerulopathy in Cfh–/– Cfd–/– mice
نویسندگان
چکیده
منابع مشابه
Circulating complement levels and C3 glomerulopathy.
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...
متن کاملSoluble CR1 therapy improves complement regulation in C3 glomerulopathy.
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...
متن کاملComplement 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...
متن کاملC3 glomerulopathy
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...
متن کاملOverview of C3 Glomerulopathy
C3 glomerulopathy is an umbrella term, which includes several rare forms of glomerulonephritis (GN) with underlying defects in the alternate complement cascade. A common histological feature noted in all these GN is dominant C3 deposition in the glomerulus. In this review, we will provide an overview of the complement system as well as mediators, with an introduction to pharmaceutical agents th...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: JCI Insight
سال: 2020
ISSN: 2379-3708
DOI: 10.1172/jci.insight.135758