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