c- Src and its role in cystic fibrosis

• Autores: Massip Copiz, María Macarena; Santa Coloma, Tomás Antonio
• Año de publicación: 2016
• Idioma: inglés
• Tipo de recurso: artículo
• Fuente: European Journal of Cell Biology Vol.95, No.10, 2016
• Materia: FIBROSIS QUISTICA; GENES; CLORURO INTRACELULAR; REGULADOR DE CONDUCTANCIA DE TRANSMEMBRANA DE FIBROSIS QUISTICA
• Institución: Pontificia Universidad Católica Argentina

Fil: Massip Copiz, María Macarena. Pontificia Universidad Católica Argentina. Facultad de Ciencias Médicas. Instituto de Investigaciones Biomédicas. Laboratorio de Biología Celular y Molecular; Argentina
Fil: Massip Copiz, María Macarena. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Santa Coloma, Tomás Antonio. Pontificia Universidad Católica Argentina. Facultad de Ciencias Médicas. Instituto de Investigaciones Biomédicas. Laboratorio de Biología Celular y Molecular; Argentina
Fil: Santa Coloma, Tomás Antonio. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Abstract: Cystic fibrosis (CF) is a lethal inherited disease produced by mutations in the gene encoding the CFTR chloride channel. Loss of function in the CFTR gene is associated with a not much noticed increased expression and activity of the non-receptor protein-tyrosine kinase c-Src. CF is therefore the result from the loss of CFTR chloride transport function and its consequences, including a chronic and excessive c-Src signaling. On the other hand, c-Src, encoded by the SRC gene, is involved in diverse signaling mechanisms that regulate key cellular functions such as cell proliferation, apoptosis, oxidative stress, inflammation, and innate immunity. These c-Src-regulated cellular functions are also affected in CF; however, studies exploring a direct role of c-Src in the regulation of these cellular functions in CF are yet scarce and often controversial. Here we describe the c-Src regulation and functions, with emphasis in those altered in CF, and describe the role of CFTR as a “signaling molecule” that negatively modulates c-Src expression and activity. It is also discussed the emerging role of intracellular Cl− and IL-1 as intermediate signaling effectors between CFTR and c-Src.