| Full text | |
| Author(s): Show less - |
Macedo, Ana Catarina Lunz
;
Lores, Lazara Elena Santisteban
;
Albuquerque, Jose Antonio Tavares
;
Duarte, Nilo Jose Coelho
;
Romano, Paschoalina
;
Ebner, Persio Almeida Rezende
;
Rezende, Vinicius Marcondes
;
Silva, Clovis A. A.
;
Andrade, Luis Eduardo Coelho
;
Vasconcelos, Dewton Moraes
;
Isaac, Lourdes
Total Authors: 11
|
| Document type: | Journal article |
| Source: | FRONTIERS IN PEDIATRICS; v. 10, p. 8-pg., 2022-11-04. |
| Abstract | |
Factor H (FH) is one of the most important regulatory proteins of the alternative pathway of the complement system. FH deficiency is a rare condition that causes unregulated C3 consumption, leading to an increased susceptibility to infections and glomerulopathies. Our previous studies have demonstrated a FH deficient patient carrying a c.452G > A, p.R127H FH mutation which leads to a misfolded protein and its retention in the endoplasmic reticulum. In his cultured fibroblasts, FH-delayed secretion was partially rescued when treated with curcumin, and once secreted, exhibited normal regulatory function. Here, we report a childhood-onset systemic lupus erythematosus (cSLE) in this FH deficient patient and the results of experimental treatment with curcumin aiming to rescue FH secretion and regulatory activity. (AU) | |
| FAPESP's process: | 17/12924-3 - Etiopathogenesis of Leptospirosis: contribution of the complement system for the control of infection in vivo and in vitro and inflammatory response: identification of gene polymorphisms of the complement system in Leptospirosis patients |
| Grantee: | Lourdes Isaac |
| Support Opportunities: | Research Projects - Thematic Grants |
| FAPESP's process: | 10/50043-0 - Complement system and pathogenicity of Leptospires: mechanisms of activation and evasion, identification of bacterial ligands, characterization of proteases and establishment of an in vivo murine model |
| Grantee: | Lourdes Isaac |
| Support Opportunities: | Research Projects - Thematic Grants |