{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Shi X"],"funding":["the 111 Project","Science and Technology Innovation Plan-Basic Research Projects","Chinese Academy of Medical Sciences","National Science Foundation of China","National Natural Science Foundation of China","the Shanghai Municipal Science and Technology Major Project"],"pagination":["e711"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC8792399"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["12(1)"],"pubmed_abstract":["Treatments for pulmonary fibrosis (PF) are ineffective because its molecular pathogenesis and therapeutic targets are unclear. Here, we show that the expression of low-density lipoprotein receptor (LDLR) was significantly decreased in alveolar type II (ATII) and fibroblast cells, whereas it was increased in endothelial cells from systemic sclerosis-related PF (SSc-PF) patients and idiopathic PF (IPF) patients compared with healthy controls. However, the plasma levels of low-density lipoprotein (LDL) increased in SSc-PF and IPF patients. The disrupted LDL-LDLR metabolism was also observed in four mouse PF models. Upon bleomycin (BLM) treatment, Ldlr-deficient (Ldlr-/-) mice exhibited remarkably higher LDL levels, abundant apoptosis, increased fibroblast-like endothelial and ATII cells and s"],"journal":["Clinical and translational medicine"],"pubmed_title":["LDLR dysfunction induces LDL accumulation and promotes pulmonary fibrosis."],"pmcid":["PMC8792399"],"funding_grant_id":["81903203","81770066","20JC1417300","2017SHZDZX01","2019‐I2M‐5‐066","31521003","2019-I2M-5-066","B13016","8203000633"],"pubmed_authors":["Zou H","Liu J","Mei X","Zuo X","Liu Q","Ma Y","Jiang S","Zhang R","Gao F","Wang J","Shi X","Zhao Y","Wang L","Ke Y","Song N","Xia J","Wang Q","Zhao S","Guo G","Sun D","Chen Y","Wu W","Tang Y","Jin L","Yin W","Yang L","Chen J","Wei D","Cui J","Zhu H","Xu J","Li Y","Huang Y","Distler JHW","Pu W","Luo R","Xu W","Tu W","Dai H"],"additional_accession":[]},"is_claimable":false,"name":"LDLR dysfunction induces LDL accumulation and promotes pulmonary fibrosis.","description":"Treatments for pulmonary fibrosis (PF) are ineffective because its molecular pathogenesis and therapeutic targets are unclear. Here, we show that the expression of low-density lipoprotein receptor (LDLR) was significantly decreased in alveolar type II (ATII) and fibroblast cells, whereas it was increased in endothelial cells from systemic sclerosis-related PF (SSc-PF) patients and idiopathic PF (IPF) patients compared with healthy controls. However, the plasma levels of low-density lipoprotein (LDL) increased in SSc-PF and IPF patients. The disrupted LDL-LDLR metabolism was also observed in four mouse PF models. Upon bleomycin (BLM) treatment, Ldlr-deficient (Ldlr-/-) mice exhibited remarkably higher LDL levels, abundant apoptosis, increased fibroblast-like endothelial and ATII cells and s","dates":{"release":"2022-01-01T00:00:00Z","publication":"2022 Jan","modification":"2026-07-16T18:44:40.96Z","creation":"2025-04-05T11:18:23.182Z"},"accession":"S-EPMC8792399","cross_references":{"pubmed":["35083881"],"doi":["10.1002/ctm2.711"]}}