A non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagy. [electronic resource]
Producer: 20150311Description: L33-47 p. digitalISSN:- 1522-1504
- ATP-Binding Cassette Transporters -- genetics
- Adaptor Proteins, Signal Transducing -- biosynthesis
- Amino Acid Substitution
- Autophagy
- Autophagy-Related Protein 8 Family
- Female
- Gene Expression Regulation -- genetics
- Genetic Diseases, Inborn -- genetics
- HEK293 Cells
- Humans
- Infant
- Lung Diseases, Interstitial -- genetics
- Lysosomes -- genetics
- Membrane Potential, Mitochondrial -- genetics
- Microfilament Proteins -- biosynthesis
- Microtubule-Associated Proteins -- biosynthesis
- Mitochondria -- genetics
- Mutation, Missense
- Proteostasis Deficiencies -- genetics
- Pulmonary Surfactant-Associated Protein C -- genetics
- Sequestosome-1 Protein
- Ubiquitin-Protein Ligases -- biosynthesis
- Vacuoles -- genetics
- rab GTP-Binding Proteins -- biosynthesis
- rab7 GTP-Binding Proteins
No physical items for this record
Publication Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
There are no comments on this title.
Log in to your account to post a comment.