NIH R01 · 2024
Molecular and Cellular Pathogenesis of Pulmonary Langerhans Cell Histiocytosis
Project Summary. Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare interstitial lung disease, which occurs almost exclusively in cigarette smokers and has a median duration of survival from of of 12.5 years. PLCH is characterized by,Langerin+ dendritic cell (DC) accumulation, inflammatory lesions, nodule formation and cystic remodeling. Recently, a causative link between acquired BRAF (kinase in RAS pathway) mutations in the myeloid/monocyte lineage and the development of benign neoplasms has been reported, and a common, acquired mutation in BRAF (V600E) was found in lung lesions of more than half of PLCH patients. Although this causal link represents an important breakthrough, the…
From the public funding record at NIH RePORTER. Describes the funded project, not the reviews below.