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Diseases of complement dysregulation-an overview.
- Source :
-
Seminars in immunopathology [Semin Immunopathol] 2018 Jan; Vol. 40 (1), pp. 49-64. Date of Electronic Publication: 2018 Jan 11. - Publication Year :
- 2018
-
Abstract
- 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.
- Subjects :
- Animals
Atypical Hemolytic Uremic Syndrome diagnosis
Atypical Hemolytic Uremic Syndrome etiology
Atypical Hemolytic Uremic Syndrome metabolism
Atypical Hemolytic Uremic Syndrome therapy
Complement Activation genetics
Complement C3 immunology
Complement C3 metabolism
Complement System Proteins genetics
Complement System Proteins metabolism
Genetic Predisposition to Disease
Glomerulonephritis etiology
Glomerulonephritis metabolism
Glomerulonephritis pathology
Hemoglobinuria, Paroxysmal diagnosis
Hemoglobinuria, Paroxysmal etiology
Hemoglobinuria, Paroxysmal metabolism
Hemoglobinuria, Paroxysmal therapy
Humans
Molecular Targeted Therapy
Phenotype
Complement Activation immunology
Complement System Proteins immunology
Disease Susceptibility immunology
Subjects
Details
- Language :
- English
- ISSN :
- 1863-2300
- Volume :
- 40
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Seminars in immunopathology
- Publication Type :
- Academic Journal
- Accession number :
- 29327071
- Full Text :
- https://doi.org/10.1007/s00281-017-0663-8