Document Detail

Complement regulatory proteins in glomerular diseases.
MedLine Citation:
PMID:  9844117     Owner:  NLM     Status:  MEDLINE    
Complement activation plays a critical role in the pathogenesis of many forms of glomerulonephritis. Complement activation leads to tissue injury through various mechanisms including the generation of chemotactic factors and activation of the resident glomerular cells following C5b-9 insertion. Recent advances have disclosed the mechanisms of regulation of complement activation by discovery of a number of complement regulatory proteins. Decay accelerating factor (DAF), membrane cofactor protein (MCP), and complement receptor type 1 (CR1) act by inactivating C3/C5 convertase. They belong to the gene superfamily known as the regulators of complement activation (RCA), and share a common structural motif called a short consensus repeat (SCR). In contrast, CD59 works by inhibiting formation of C5b-9. The glomerulus is particularly well endowed with these membrane-bound complement regulatory proteins. DAF, MCP, and CD59 are ubiquitously expressed by all three resident glomerular cells, while CR1 is localized exclusively in podocytes. Expression of complement regulatory proteins can be changed by many factors including complement attack itself, and their expression levels are affected in various glomerular disorders. Studies utilizing cultured glomerular cells and animal models of glomerular diseases suggest important protective roles of complement regulatory proteins against immune-mediated renal injury. Recent progress in molecular biological techniques has made new therapeutic strategy feasible. Systemic administration of soluble recombinant complement regulatory proteins and local overexpression of complement regulatory proteins are promising therapeutic approaches.
M Nangaku
Related Documents :
4595567 - Coordinate inhibition of elongation factor g function and ribosomal subunit association...
10805567 - Structural analysis of the elongation factor g protein from the low-temperature-adapted...
11601697 - Chicken mim-1 protein, p33, is a heterophil chemotactic factor present in salmonella en...
9436907 - Escherichia coli release factor 3: resolving the paradox of a typical g protein structu...
12421697 - Hephaestus encodes a polypyrimidine tract binding protein that regulates notch signalli...
18094607 - Roles of escrt in autophagy-associated neurodegeneration.
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Kidney international     Volume:  54     ISSN:  0085-2538     ISO Abbreviation:  Kidney Int.     Publication Date:  1998 Nov 
Date Detail:
Created Date:  1999-02-11     Completed Date:  1999-02-11     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0323470     Medline TA:  Kidney Int     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1419-28     Citation Subset:  IM    
Division of Nephrology and Endocrinology, University of Tokyo School of Medicine, Tokyo, Japan.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Antigens, CD / physiology
Antigens, CD46
Antigens, CD55 / physiology
Antigens, CD59 / physiology
Complement Activation*
Glomerulonephritis / immunology*,  therapy
Membrane Glycoproteins / physiology
Receptors, Complement / physiology
Receptors, Complement 3b / physiology
Reg. No./Substance:
0/Antigens, CD; 0/Antigens, CD46; 0/Antigens, CD55; 0/Antigens, CD59; 0/CD46 protein, human; 0/Membrane Glycoproteins; 0/Receptors, Complement; 0/Receptors, Complement 3b

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Hypophosphatemic rickets accompanying congenital microvillous atrophy.
Next Document:  Is complement a target for therapy in renal disease?