Chronic Expression of a Clinical SFTPC Mutation Causes Murine Lung Fibrosis with Idiopathic Pulmonary Fibrosis Features

January 1, 2023
Am J Respir Cell Mol Biol
https://pubmed.ncbi.nlm.nih.gov/36473455

This article is currently being updated. View its version on PubMed.

https://pubmed.ncbi.nlm.nih.gov/36473455

Research summary

This study developed a mouse model expressing the human SFTPC C121G mutation linked to interstitial lung disease. The model develops spontaneous lung fibrosis and replicates key pathological features of idiopathic pulmonary fibrosis (IPF), including AEC2 cell dysfunction and ER stress.

Key outcome of the study

Expression of the C121G mutation leads to spontaneous, progressive lung fibrosis and impaired lung function, modeling IPF pathology.

Mouse model

Tamoxifen-inducible Sftpc(C121G) Knockin mouse model with AEC2-specific expression of the mutation.

TARGET:
SFTPC
Surfactant Protein C

Keywords

Idiopathic pulmonary fibrosis, Lung fibrosis, ER stress, Alveolar epithelium

Technical specifications

Knockin, Tamoxifen-inducible, Point mutation, AEC2-specific expression, Rosa26

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Time-dependent KO mouse

Use an inducible conditional Knockout mouse to age-dependently inactivate your gene, and to enable studies at defined development stages or on age-related diseases.