Jiang, Xu

Chemical chaperone 4-phenylbutyric acid alleviates the aggregation of human familial pulmonary fibrosis-related mutant SP-A2 protein in part through effects on GRP78. [electronic resource] - Biochimica et biophysica acta. Molecular basis of disease 10 2018 - 3546-3557 p. digital

Publication Type: Journal Article; Research Support, Non-U.S. Gov't

1879-260X

10.1016/j.bbadis.2018.08.014 doi


A549 Cells
Animals
CHO Cells
Cricetulus
Dose-Response Relationship, Drug
Endoplasmic Reticulum Chaperone BiP
Heat-Shock Proteins--metabolism
Humans
Leupeptins--pharmacology
Mutation
Phenylbutyrates--pharmacology
Protein Aggregates--drug effects
Pulmonary Fibrosis--genetics
Pulmonary Surfactant-Associated Protein A--genetics