Can correcting the ΔF508-CFTR proteostasis-defect rescue CF lung disease?

Current Molecular Medicine

Volumn 12, Issue 7, 2012, Pages 860-871

  Valle, C W ^a   Vij, N ^a  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

CFTR; Cystic fibrosis; ERAD; Proteasome; Proteostasis; Therapeutics; Ubiquitin; F508

Indexed keywords

BORTEZOMIB; CALNEXIN; DELTA F508 TRANSMEMBRANE CONDUCTANCE REGULATOR; DYNAMIN; GUANOSINE TRIPHOSPHATASE; HEAT SHOCK PROTEIN 40; HEAT SHOCK PROTEIN 70; HEAT SHOCK PROTEIN 90; IMMUNOGLOBULIN ENHANCER BINDING PROTEIN; LECTIN; MIGLUSTAT; MYOSIN VI; PHENYLALANINE; PROTEIN P97; RAB PROTEIN; RAB11 PROTEIN; RAB7 PROTEIN; SILDENAFIL; TRANSCRIPTION FACTOR AP 2; TRANSMEMBRANE CONDUCTANCE REGULATOR; UNCLASSIFIED DRUG;

APICAL MEMBRANE; ARTICLE; CELL FATE; CELL MEMBRANE; CELL MIGRATION; CELL SURFACE; CHLORIDE TRANSPORT; CHRONIC OBSTRUCTIVE LUNG DISEASE; CONCENTRATION (PARAMETERS); CYSTIC FIBROSIS; DEACETYLATION; ENDOCYTOSIS; ENDOPLASMIC RETICULUM; ENDOPLASMIC RETICULUM ASSOCIATED DEGRADATION; ENDOSOME; GENE DELETION; GENE MUTATION; GLYCOSYLATION; GOLGI COMPLEX; HUMAN; INTERNALIZATION; ION TRANSPORT; LUNG ALVEOLUS EPITHELIUM; LUNG FUNCTION TEST; NONHUMAN; PROTEIN DEGRADATION; PROTEIN FOLDING; PROTEIN HOMEOSTASIS; PROTEIN LOCALIZATION; PROTEIN PROCESSING; RESPIRATORY TRACT INFLAMMATION; THERMODYNAMICS;

EID: 84863523203     PISSN: 15665240     EISSN: 18755666     Source Type: Journal    
DOI: 10.2174/156652412801318773     Document Type: Article

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