National Heart, Lung, and Blood Institute 5R01HL162662-04

Molecular and Cellular Pathogenesis of Pulmonary Langerhans Cell Histiocytosis

NIH awarded $530,966 to University of Cincinnati for research on Pulmonary Langerhans Cell Histiocytosis, focusing on BRAF mutations and cigarette smoke effects to develop preclinical models and identify therapeutic targets.

Description

This project aims to develop preclinical models that mimic hallmark features of Pulmonary Langerhans Cell Histiocytosis (PLCH), focusing on the role of BRAF mutations and cigarette smoke exposure in disease initiation and progression. The research hypothesizes that BRAF mutations, combined with cigarette smoke, amplify CCL20-mediated histiocyte accumulation, leading to inflammatory nodule formation via CCL7 production, with BRAF-dependent expression of immune evasion ligands further perpetuating disease. The study will test this hypothesis through three specific aims, with the goal of advancing understanding of PLCH pathogenesis and identifying potential therapeutic targets. The project provides a platform for preclinical studies and future clinical trials, aiming to develop effective treatments for this life-threatening pulmonary disease.

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