TY  - GEN
AU  - Hawkins,Arie
AU  - Guttentag,Susan H
AU  - Deterding,Robin
AU  - Funkhouser,William K
AU  - Goralski,Jennifer L
AU  - Chatterjee,Shampa
AU  - Mulugeta,Surafel
AU  - Beers,Michael F
TI  - A non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagy
SN  - 1522-1504
PY  - 2015///0311
KW  - ATP-Binding Cassette Transporters
KW  - genetics
KW  - Adaptor Proteins, Signal Transducing
KW  - biosynthesis
KW  - Amino Acid Substitution
KW  - Autophagy
KW  - Autophagy-Related Protein 8 Family
KW  - Female
KW  - Gene Expression Regulation
KW  - Genetic Diseases, Inborn
KW  - HEK293 Cells
KW  - Humans
KW  - Infant
KW  - Lung Diseases, Interstitial
KW  - Lysosomes
KW  - Membrane Potential, Mitochondrial
KW  - Microfilament Proteins
KW  - Microtubule-Associated Proteins
KW  - Mitochondria
KW  - Mutation, Missense
KW  - Proteostasis Deficiencies
KW  - Pulmonary Surfactant-Associated Protein C
KW  - Sequestosome-1 Protein
KW  - Ubiquitin-Protein Ligases
KW  - Vacuoles
KW  - rab GTP-Binding Proteins
KW  - rab7 GTP-Binding Proteins
N1  - 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
UR  - https://doi.org/10.1152/ajplung.00217.2014
ER  -