8
IRUS Total
Downloads

C3 dysregulation due to factor H deficiency is mannan-binding lectin-associated serine proteases (MASP)-1 and MASP-3 independent in vivo

Title: C3 dysregulation due to factor H deficiency is mannan-binding lectin-associated serine proteases (MASP)-1 and MASP-3 independent in vivo
Authors: Ruseva, MM
Takahashi, M
Fujita, T
Pickering, MC
Item Type: Journal Article
Abstract: Uncontrolled activation of the complement alternative pathway is associated with complement‐mediated renal disease. Factor B and factor D are essential components of this pathway, while factor H (FH) is its major regulator. In complete FH deficiency, uncontrolled C3 activation through the alternative pathway results in plasma C3 depletion and complement‐mediated renal disease. These are dependent on factor B. Mannan‐binding lectin‐associated serine proteases 1 and 3 (MASP‐1, MASP‐3) have been shown recently to contribute to alternative pathway activation by cleaving pro‐factor D to its active form, factor D. We studied the contribution of MASP‐1 and MASP‐3 to uncontrolled alternative pathway activation in experimental complete FH deficiency. Co‐deficiency of FH and MASP‐1/MASP‐3 did not ameliorate either the plasma C3 activation or glomerular C3 accumulation in FH‐deficient mice. Our data indicate that MASP‐1 and MASP‐3 are not essential for alternative pathway activation in complete FH deficiency.
Issue Date: 1-Apr-2014
Date of Acceptance: 20-Nov-2013
URI: http://hdl.handle.net/10044/1/87721
DOI: 10.1111/cei.12244
ISSN: 0009-9104
Publisher: Wiley
Start Page: 84
End Page: 92
Journal / Book Title: Clinical and Experimental Immunology
Volume: 176
Issue: 1
Copyright Statement: © 2013 The Authors. Clinical and Experimental Immunology published by John Wiley & Sons Ltd on behalf of British Society for Immunology. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Sponsor/Funder: Wellcome Trust
Funder's Grant Number: 098476/Z/12/Z
Keywords: Science & Technology
Life Sciences & Biomedicine
Immunology
MASP-1/3
complement
kidney
ALTERNATIVE COMPLEMENT PATHWAY
HUMAN SERUM
NOR MASP-3
MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS
FACTOR-B
ACTIVATION
GLOMERULOPATHY
MUTATIONS
MECHANISM
ZYMOGEN
MASP-1/3
complement
kidney
Animals
Blotting, Western
Complement Activation
Complement C3
Complement C5
Complement Factor B
Complement Factor D
Complement Factor H
Complement Pathway, Alternative
Enzyme-Linked Immunosorbent Assay
Glomerular Basement Membrane
Hereditary Complement Deficiency Diseases
Kidney Diseases
Kidney Glomerulus
Mannose-Binding Protein-Associated Serine Proteases
Mice
Mice, Inbred C57BL
Mice, Knockout
Kidney Glomerulus
Animals
Mice, Inbred C57BL
Mice, Knockout
Mice
Kidney Diseases
Complement Factor B
Complement Factor D
Complement Factor H
Blotting, Western
Enzyme-Linked Immunosorbent Assay
Complement Activation
Complement Pathway, Alternative
Complement C3
Complement C5
Glomerular Basement Membrane
Mannose-Binding Protein-Associated Serine Proteases
Hereditary Complement Deficiency Diseases
Science & Technology
Life Sciences & Biomedicine
Immunology
MASP-1/3
complement
kidney
ALTERNATIVE COMPLEMENT PATHWAY
HUMAN SERUM
NOR MASP-3
MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS
FACTOR-B
ACTIVATION
GLOMERULOPATHY
MUTATIONS
MECHANISM
ZYMOGEN
1107 Immunology
Immunology
Publication Status: Published
Online Publication Date: 2013-11-26
Appears in Collections:Department of Immunology and Inflammation



This item is licensed under a Creative Commons License Creative Commons