scholarly journals Kidney Diseases Associated With Alternative Complement Pathway Dysregulation and Potential Treatment Options

2017 ◽  
Vol 354 (6) ◽  
pp. 533-538 ◽  
Author(s):  
Prateek Sanghera ◽  
Mythili Ghanta ◽  
Fatih Ozay ◽  
Venkatesh K. Ariyamuthu ◽  
Bekir Tanriover
2014 ◽  
Vol 24 (5) ◽  
pp. 326-335 ◽  
Author(s):  
Scott P. Henry ◽  
Mark A. Jagels ◽  
Tony E. Hugli ◽  
Sheri Manalili ◽  
Richard S. Geary ◽  
...  

2021 ◽  
Vol 12 ◽  
Author(s):  
Jonathan Barratt ◽  
Ilene Weitz

The complement system is central to first-line defense against invading pathogens. However, excessive complement activation and/or the loss of complement regulation contributes to the development of autoimmune diseases, systemic inflammation, and thrombosis. One of the three pathways of the complement system, the alternative complement pathway, plays a vital role in amplifying complement activation and pathway signaling. Complement factor D, a serine protease of this pathway that is required for the formation of C3 convertase, is the rate-limiting enzyme. In this review, we discuss the function of factor D within the alternative pathway and its implication in both healthy physiology and disease. Because the alternative pathway has a role in many diseases that are characterized by excessive or poorly mediated complement activation, this pathway is an enticing target for effective therapeutic intervention. Nonetheless, although the underlying disease mechanisms of many of these complement-driven diseases are quite well understood, some of the diseases have limited treatment options or no approved treatments at all. Therefore, in this review we explore factor D as a strategic target for advancing therapeutic control of pathological complement activation.


2003 ◽  
Vol 10 (2) ◽  
pp. 216-220
Author(s):  
Marlene Pereira de Carvalho Florido ◽  
Patrícia Ferreira de Paula ◽  
Lourdes Isaac

ABSTRACT Due to the increasing numbers of reported clinical cases of complement deficiency in medical centers, clinicians are now more aware of the role of the complement system in the protection against infections caused by microorganisms. Therefore, clinical laboratories are now prepared to perform a number of diagnostic tests of the complement system other than the standard 50% hemolytic component assay. Deficiencies of alternative complement pathway proteins are related to severe and recurrent infections; and the application of easy, reliable, and low-cost methods for their detection and distinction are always welcome, notably in developing countries. When activation of the alternative complement pathway is evaluated in hemolytic agarose plates, some but not all human sera cross-react to form a late linear lysis. Since the formation of this linear lysis is dependent on C3 and factor B, it is possible to use late linear lysis to routinely screen for the presence of deficiencies of alternative human complement pathway proteins such as factor B. Furthermore, since linear lysis is observed between normal human serum and primary C3-deficient serum but not between normal human serum and secondary C3-deficient serum caused by the lack of factor H or factor I, this assay may also be used to discriminate between primary and secondary C3 deficiencies.


1988 ◽  
Vol 23 (4) ◽  
pp. 213-217 ◽  
Author(s):  
Tomoki YANO ◽  
Kazuhiro FUJIKI ◽  
Miki NAKAO ◽  
Hiroko MATSUYAMA

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