Proteomics of hosts and pathogens in cystic fibrosis

Abstract

Cystic fibrosis (CF) is a congenital disease that results in great morbidity and mortality mainly in the Caucasian population. Although CF is a monogenic disease caused by mutation in the CF conductance transmembrane regulator (CFTR) gene, most of the related mortality can be attributed to infection mediated by opportunistic bacterial and fungal pathogens. Over the past decade, advancements in the field of proteomics have helped to gain insight into the repertoire of host and pathogen proteins involved in CF pathophysiology. This review provides an overview of the contributions of proteomic studies in advancing our knowledge of the biology of CF and disease progression associated with pathogen infection and host defense responses.

Bactericidal and Fungicidal Activity of N -Chlorotaurine Is Enhanced in Cystic Fibrosis Sputum Medium

“…They can persist for a long time, i.e., 6 to 70 months, in the CF lung and thereby adapt to their specific environ-ment (7,8). Staphylococcus aureus, for instance, forms small colony variants that may evade the defense system and are associated with greater resistance to antibiotics (9).…”

“…As a consequence, methicillin-resistant S. aureus (MRSA) emerges, followed by P. aeruginosa, which typically forms alginate biofilms and frequently displays multidrug resistance (4). Besides these prevailing pathogens, numerous other bacteria and also fungi have been associated with infections (4,8). Regarding the latter, filamentous fungi, mainly Aspergillus fumigatus, Scedosporium apiospermum, and Aspergillus terreus, have been associated with deterioration of lung function (10)(11)(12); Candida albicans is thought to be mostly commensal (11), although it may cause allergic bronchopulmonary mycosis or disseminated mycosis in immunosuppressed patients (13).…”

Frecuencia de las mutaciones más comunes del gen CFTR en pacientes peruanos con fibrosis quística mediante la técnica ARMS-PCR

“…La fibrosis quística (FQ) es una enfermedad congénita ocasionada por un gen autosómico recesivo que afecta a 1 de cada 3 500 nacidos vivos a nivel mundial y cuya prevalencia es mayor entre poblaciones caucásicas (1) . Las mutaciones que inducen la FQ han sido asociadas a defectos de una proteína reguladora del transporte de cloro codificada por el gen CFTR (en inglés: Cystic fibrosis transmembrane conductance regulator) (2) .…”

“…Las mutaciones en el CFTR causan un defecto en el transporte de agua dentro y fuera de las células, lo que resulta en exceso de secreción de moco anormalmente espeso y viscoso a nivel pulmonar, pancreático, e intestinal, y conlleva a complicaciones graves e incluso la muerte (1) . A la fecha, más de 1 900 mutaciones diferentes han sido identificadas en el gen CFTR, sin embargo, la deleción del codón para la fenilalanina en la posición 508 (p.Phe508del) es la más frecuente (1) .…”

“…A la fecha, más de 1 900 mutaciones diferentes han sido identificadas en el gen CFTR, sin embargo, la deleción del codón para la fenilalanina en la posición 508 (p.Phe508del) es la más frecuente (1) .…”