Endoplasmic reticulum stress, a new wrestler, in the pathogenesis of idiopathic pulmonary fibrosis.
Idiopathic pulmonary fibrosis (IPF) has attracted extensive attention for its unexplained progressive lung scarring, short median survival and its unresponsiveness to traditional therapies. Despite extensive studies, the mechanisms underlying IPF pathoetiologies, however, remain poorly understood. Recent advances delineated a potential function of endoplasmic reticulum (ER) stress in meeting the need of fibrotic response, which pinpointed a critical role for the unfolded protein response (UPR) pathways in IPF pathogenesis. In this review, we highlight the effect of ER stress and the activation of UPR on the survival, differentiation, function and proliferation of major profibrotic cells in lung tissues during the course of IPF, and discuss the feasibility whether targeting UPR components could be an orientation for developing effective therapeutic strategies against this devastating disorder in clinical settings.
The Center for Biomedical Research, Key Laboratory of Organ Transplantation, Ministry of Education, Ministry of Health, Tongji Hospital, Tongji Medical College, Huazhong University of Science & Technology Wuhan, China.
Lei
L
Zhang
The Center for Biomedical Research, Key Laboratory of Organ Transplantation, Ministry of Education, Ministry of Health, Tongji Hospital, Tongji Medical College, Huazhong University of Science & Technology Wuhan, China.
Yi
Y
Wang
The Center for Biomedical Research, Key Laboratory of Organ Transplantation, Ministry of Education, Ministry of Health, Tongji Hospital, Tongji Medical College, Huazhong University of Science & Technology Wuhan, China.
Nuruliarizki Shinta
NS
Pandupuspitasari
The Center for Biomedical Research, Key Laboratory of Organ Transplantation, Ministry of Education, Ministry of Health, Tongji Hospital, Tongji Medical College, Huazhong University of Science & Technology Wuhan, China.
Guorao
G
Wu
Department of Emergency Medicine, Institute of Emergency Medicine and Rare Diseases, The Second Xiangya Hospital, Central South University Changsha, China.
Xudong
X
Xiang
Clinical and Molecular Immunology Research Center, Department of Immunology, Medical College of Yangtze University Jingzhou, Hubei, China.
Quan
Q
Gong
The Center for Biomedical Research, Key Laboratory of Organ Transplantation, Ministry of Education, Ministry of Health, Tongji Hospital, Tongji Medical College, Huazhong University of Science & Technology Wuhan, China.
Weining
W
Xiong
The Center for Biomedical Research, Key Laboratory of Organ Transplantation, Ministry of Education, Ministry of Health, Tongji Hospital, Tongji Medical College, Huazhong University of Science & Technology Wuhan, China.
Cong-Yi
CY
Wang
The Center for Biomedical Research, Key Laboratory of Organ Transplantation, Ministry of Education, Ministry of Health, Tongji Hospital, Tongji Medical College, Huazhong University of Science & Technology Wuhan, China.
Ping
P
Yang
Clinical and Molecular Immunology Research Center, Department of Immunology, Medical College of Yangtze University Jingzhou, Hubei, China.
Boxu
B
Ren
ER stress; IPF; UPR; pathogenesis; profibrotic cells