A non-BRICHOS SFTPC mutant (SP-CI73T) linked to interstitial lung disease promotes a late block in macroautophagy disrupting cellular proteostasis and mitophagy

American Journal of Physiology - Lung Cellular and Molecular Physiology

Volumn 308, Issue 1, 2015, Pages L33-L47

  Hawkins, Arie ^a   Guttentag, Susan H ^b   Deterding, Robin ^c   Funkhouser, William K ^d   Goralski, Jennifer L ^d   Chatterjee, Shampa ^e   Mulugeta, Surafel ^a   Beers, Michael F ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b MONROE CARELL JR CHILDREN S HOSPITAL AT VANDERBILT   (United States)

^c Children's Hospital Colorado   (United States)

^d UNIVERSITY OF NORTH CAROLINA SCHOOL OF MEDICINE   (United States)

^e UNIVERSITY OF PENNSYLVANIA   (United States)

Author keywords

Alveolar epithelium; Amphisome; Autophagy; Pulmonary fibrosis; Surfactant protein

Indexed keywords

MICROTUBULE ASSOCIATED PROTEIN 1; SURFACTANT PROTEIN C; ABC TRANSPORTER; ABCA3 PROTEIN, HUMAN; ACTIN BINDING PROTEIN; GABARAPL2 PROTEIN, HUMAN; LIGHT CHAIN 3, HUMAN; MICROTUBULE ASSOCIATED PROTEIN; PARKIN; RAB PROTEIN; RAB7 PROTEIN; SFTPC PROTEIN, HUMAN; SIGNAL TRANSDUCING ADAPTOR PROTEIN; SQSTM1 PROTEIN, HUMAN; UBIQUITIN PROTEIN LIGASE;

ALLELE; ARTICLE; AUTOPHAGOSOME; BIOENERGY; CASE REPORT; CELL HYPERPLASIA; CELL LYSATE; CELL MATURATION; CELL MEMBRANE; CELL ULTRASTRUCTURE; CELL VACUOLE; CELLULAR DISTRIBUTION; CHILD; ECZEMA; ELECTROPHORETIC MOBILITY; FAILURE TO THRIVE; FEMALE; FLUORESCENCE MICROSCOPY; GENE EXPRESSION; GENE PRODUCT; GENETIC SCREENING; HEK293 CELL LINE; HUMAN; HUMAN CELL; INTERSTITIAL LUNG DISEASE; LUNG ALVEOLUS CELL TYPE 2; LUNG ALVEOLUS MACROPHAGE; LUNG BIOPSY; MACROAUTOPHAGY; MISSENSE MUTATION; MITOCHONDRIAL DYNAMICS; MITOCHONDRIAL MEMBRANE; MITOPHAGY; MULTIVESICULAR BODY; PHENOTYPE; PRESCHOOL CHILD; PROTEIN HOMEOSTASIS; PROTEIN PROCESSING; STABLE EXPRESSION; TRANSIENT EXPRESSION; TRANSMISSION ELECTRON MICROSCOPY; UPPER RESPIRATORY TRACT INFECTION; WESTERN BLOTTING; AMINO ACID SUBSTITUTION; AUTOPHAGY; BIOSYNTHESIS; GENE EXPRESSION REGULATION; GENETIC DISORDER; GENETICS; INFANT; LYSOSOME; METABOLISM; MITOCHONDRIAL MEMBRANE POTENTIAL; MITOCHONDRION; PATHOLOGY; PROTEOSTASIS DEFICIENCY; ULTRASTRUCTURE;

ADAPTOR PROTEINS, SIGNAL TRANSDUCING; AMINO ACID SUBSTITUTION; ATP-BINDING CASSETTE TRANSPORTERS; AUTOPHAGY; FEMALE; GENE EXPRESSION REGULATION; GENETIC DISEASES, INBORN; HEK293 CELLS; HUMANS; INFANT; LUNG DISEASES, INTERSTITIAL; LYSOSOMES; MEMBRANE POTENTIAL, MITOCHONDRIAL; MICROFILAMENT PROTEINS; MICROTUBULE-ASSOCIATED PROTEINS; MITOCHONDRIA; MUTATION, MISSENSE; PROTEOSTASIS DEFICIENCIES; PULMONARY SURFACTANT-ASSOCIATED PROTEIN C; RAB GTP-BINDING PROTEINS; UBIQUITIN-PROTEIN LIGASES; VACUOLES;

EID: 84920270297     PISSN: 10400605     EISSN: 15221504     Source Type: Journal    
DOI: 10.1152/ajplung.00217.2014     Document Type: Article

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