期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 21, 期 9, 页码 -出版社
MDPI
DOI: 10.3390/ijms21093331
关键词
cystic fibrosis; inflammation; immunotherapy; lung disease; proteases
资金
- UAB Research Development Program Postdoctoral Fellowship
- US CF Foundation Research Development Program [ROWE19R0]
- National Institutes of Health (NIH, USA) [R01 HL102371]
- US CF Foundation Basic Research Program [TIROUV17G0, TIROUV19G0]
Cystic fibrosis (CF) lung disease is characterized by unconventional mechanisms of inflammation, implicating a chronic immune response dominated by innate immune cells. Historically, therapeutic development has focused on the mutated cystic fibrosis transmembrane conductance regulator (CFTR), leading to the discovery of small molecules aiming at modulating and potentiating the presence and activity of CFTR at the plasma membrane. However, treatment burden sustained by CF patients, side effects of current medications, and recent advances in other therapeutic areas have highlighted the need to develop novel disease targeting of the inflammatory component driving CF lung damage. Furthermore, current issues with standard treatment emphasize the need for directed lung therapies that could minimize systemic side effects. Here, we summarize current treatment used to target immune cells in the lungs, and highlight potential benefits and caveats of novel therapeutic strategies.
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