| Autor: |
Nicholas R. Medjeral-Thomas, Anne Troldborg, Nicholas Constantinou, Hannah J. Lomax-Browne, Annette G. Hansen, Michelle Willicombe, Charles D. Pusey, H. Terence Cook, Steffen Thiel, Matthew C. Pickering |
| Jazyk: |
angličtina |
| Rok vydání: |
2018 |
| Předmět: |
|
| Zdroj: |
Kidney International Reports, Vol 3, Iss 2, Pp 426-438 (2018) |
| Druh dokumentu: |
article |
| ISSN: |
2468-0249 |
| DOI: |
10.1016/j.ekir.2017.11.015 |
| Popis: |
IgA nephropathy (IgAN) is characterized by glomerular deposition of galactose-deficient IgA1 and complement proteins and leads to renal impairment. Complement deposition through the alternative and lectin activation pathways is associated with renal injury. Methods: To elucidate the contribution of the lectin pathway to IgAN, we measured the 11 plasma lectin pathway components in a well-characterized cohort of patients with IgAN. Results: M-ficolin, L-ficolin, mannan-binding lectin (MBL)–associated serine protease (MASP)-1 and MBL-associated protein (MAp) 19 were increased, whereas plasma MASP-3 levels were decreased in patients with IgAN compared with healthy controls. Progressive disease was associated with low plasma MASP-3 levels and increased glomerular staining for C3b/iC3b/C3c, C3d, C4d, C5b-9, and factor H–related protein 5 (FHR5). Glomerular FHR5 deposition positively correlated with glomerular C3b/iC3b/C3c, C3d, and C5b-9 deposition, but not with glomerular C4d. These observations, together with the finding that glomerular factor H (fH) deposition was reduced in progressive disease, are consistent with a role for fH deregulation by FHR5 in renal injury in IgAN. Conclusion: Our data indicate that circulating MASP-3 levels could be used as a biomarker of disease severity in IgAN and that glomerular staining for FHR5 could both indicate alternative complement pathway activation and be a tissue marker of disease severity. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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