+ Site Statistics
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

Diseases of complement dysregulation-an overview

Diseases of complement dysregulation-an overview

Seminars in Immunopathology 40(1): 49-64

Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease. Eculizumab has revolutionized the treatment of PNH and aHUS although has been less successful in C3G. With the next generation of complement therapeutic in late stage development, these archetypal complement diseases will provide the initial targets.

(PDF emailed within 0-6 h: $19.90)

Accession: 058427381

Download citation: RISBibTeXText

PMID: 29327071

DOI: 10.1007/s00281-017-0663-8

Related references

Overview of Laboratory Testing and Clinical Presentations of Complement Deficiencies and Dysregulation. Advances in Clinical Chemistry 77: 1-75, 2017

Kidney diseases caused by complement dysregulation: acquired, inherited, and still more to come. Clinical and Developmental Immunology 2012: 695131, 2013

Kidney Diseases Associated With Alternative Complement Pathway Dysregulation and Potential Treatment Options. American Journal of the Medical Sciences 354(6): 533-538, 2017

Complement 3 + -astrocytes are highly abundant in prion diseases, but their abolishment led to an accelerated disease course and early dysregulation of microglia. Acta Neuropathologica Communications 7(1): 83, 2019

Therapeutic Plasma Exchange Strategy in Complement-Mediated Diseases: An Overview. Therapeutic Apheresis and Dialysis 22(1): 8-10, 2018

Decreased Serum Levels of Complement C3 Reflect Complement System Dysregulation in Patients With Primary Open-angle Glaucoma: Results From a Pilot Study. Journal of Glaucoma 27(9): 761-768, 2018

Studies on the complement c 3 and complement c 4 components of human complement in a healthy population and patients with various diseases. Zeitschrift fuer Klinische Chemie und Klinische Biochemie 13(6): 258-259, 1975

The eye as a complement dysregulation hotspot. Seminars in Immunopathology 40(1): 65-74, 2017

Atypical HUS and complement dysregulation. Journal of the American Society of Nephrology 17(7): 1775-1776, 2006

Inherited dysregulation of the complement system. Bulletin et Memoires de l'Academie Royale de Medecine de Belgique 159(Pt 2): 195-198, 2004

The role of complement dysregulation in AMD mouse models. Advances in Experimental Medicine and Biology 801: 213-219, 2014

Dysregulation of the complement system in neurodegenerative disease. Molecular Immunology 47(13): 2270-2270, 2010

Preeclampsia is characterized by placental complement dysregulation. Hypertension 60(5): 1332-1337, 2013

Complement dysregulation in AMD: RPE-Bruch's membrane-choroid. Molecular Aspects of Medicine 33(4): 436-445, 2012

Complement dysregulation in haemolytic uraemic syndrome. Lancet 362(9395): 1514-1515, 2003