학술논문

The complement alternative pathway in health and disease—activation or amplification?
Document Type
Article
Source
Immunological Reviews. Jan2023, Vol. 313 Issue 1, p6-14. 9p.
Subject
*PAROXYSMAL hemoglobinuria
*REPERFUSION injury
*COMPLEMENT factor H
*PATHOLOGY
*MACULAR degeneration
*ANTINEUTROPHIL cytoplasmic antibodies
*MEDICAL research
Language
ISSN
0105-2896
Abstract
The roles of the AP/AL in both healthy individuals and disease are highlighted in this issue of Immunological Reviews and take us on a journey from the initial tickover hypothesis through understanding of basic mechanistic and disease pathology, something that continues today, to seminal and ground-breaking therapeutic strategies. Beginning with an overview of both hemostasis and amplified C3 activation, Noris and Galbusera drive home the inter-relationship of the two systems, and its malfunction in thrombotic conditions.[24] One interesting aspect of their discussion is that thrombotic thrombocytopenic purpura (TTP), a disease not commonly regarded as a disease of dysregulated complement activation, has an AP/AL activation component. In addition to these fulminant diseases caused by dysregulation of the AP/AL, more subtle functional defects in AP/AL regulation contribute to common diseases, including age-related macular degeneration. In late 2020, one of us (RAH) was approached by the late Professor Sir Peter Lachmann and invited to join him in co-editing a collection of articles focused on the alternative pathway (AP), or amplification loop (AL), of C3 activation. [Extracted from the article]