Article
MAPK mutations and cigarette smoke promote the pathogenesis of pulmonary Langerhans cell histiocytosis.
JCI insight - 27 Feb 2020
Liu Huan, Osterburg Andrew R, Flury Jennifer, Swank Zulma, McGraw Dennis W, Gupta Nishant, Wikenheiser-Brokamp Kathryn A, Kumar Ashish, Tazi Abdellatif, Inoue Yoshikazu, Hirose Masaki, McCormack Francis X, Borchers Michael T
Abstract excerpt
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare smoking-related lung disease characterized by dendritic cell (DC) accumulation, bronchiolocentric nodule formation, and cystic lung remodeling. Approximately 50% of patients with PLCH harbor somatic BRAF-V600E mutations in cells of the myeloid/monocyte lineage. However, the rarity of the disease and lack of animal models have impeded the study of PLCH...
Topics
- Animals
- CD11c Antigen
- Disease Models, Animal
- Histiocytosis, Langerhans-Cell
- Lung Diseases
- Mice
- Mitogen-Activated Protein Kinases
- Mutation
- Proto-Oncogene Proteins B-raf
- Smoke