TGF- β1 Regulates Peroxisomal Genes/Proteins via Smad Signaling in Idiopathic Pulmonary Fibrosis Fibroblasts and Transgenic Mouse Models

Idiopathic pulmonary fibrosis (IPF) is a chronic human disease with persistent destruction of lung parenchyma. A pivotal role in the initiation and pathogenesis of IPF plays the TGF- β1 signaling. As shown here, TGF-β1 signaling downregulates not only peroxisome biogenesis but also the metabolism of these organelles in human IPF fibroblasts. In accordance with the in vitro cell culture observations in human fibroblasts and human lung tissue, peroxisomal biogenesis and metaboli c proteins were significantly downregulated in the lung of one-month-old transgenic mice expressing a constitutively active TGF-β type I receptor (TβRI/ALK5).
Source: American Journal of Pathology - Category: Pathology Authors: Tags: Regular Article Source Type: research