Book chapter
The Nature of the Diseases That Arise from Improper Regulation of the Alternative Pathway of Complement
Manual of Molecular and Clinical Laboratory Immunology, pp.138-144
ASM Press
05/11/2016
DOI: 10.1128/9781555818722.ch15
Abstract
The complement system is the cornerstone of innate immunity. As one of the first lines of host defense, it plays a major role in microbial killing, immune complex handling, apoptotic cell clearance, tissue homeostasis, and modulation of adaptive immunity (1–3). Critical to these functions is the sequential triggering of a series of cascades that result in the generation of metastable protease complexes and culminate in the formation of membrane attack complex (MAC) (4). Improper regulation of these cascades is associated with the development of multiple different diseases. In this chapter, we focus on the clinical consequence of dysregulation of the alternative pathway (AP) of complement. We first provide a review of the AP and then illustrate the consequence of its dysregulation by describing two ultrarare diseases: atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G).
Details
- Title: Subtitle
- The Nature of the Diseases That Arise from Improper Regulation of the Alternative Pathway of Complement
- Creators
- Richard J. H Smith - University of Iowa
- Contributors
- Barbara Detrick (Editor)John L Schmitz (Editor)Robert G Hamilton (Editor)
- Resource Type
- Book chapter
- Publication Details
- Manual of Molecular and Clinical Laboratory Immunology, pp.138-144
- DOI
- 10.1128/9781555818722.ch15
- Publisher
- ASM Press; Washington, DC, USA
- Number of pages
- 7
- Language
- English
- Date published
- 05/11/2016
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
- Record Identifier
- 9984256793702771
Metrics
17 Record Views