A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnover

Human molecular genetics

Volumn 23, Issue 19, 2014, Pages 5109-5122

  Long, Olivia S ^a   Benson, Joshua A ^a   Kwak, Joon H yeok ^a   Luke, Cliff J ^a   Gosai, Sager J ^a   O'Reilly, Linda P ^a   Wang, Yan ^a   Li, Jie ^a   Vetica, Anne C ^a   Miedel, Mark T ^a   Stolz, Donna B ^a   Watkins, Simon C ^a   Züchner, Stephan ^a   Perlmutter, David H ^a   Silverman, Gary A ^a   Pak, Stephen C ^a  

^a CHILDREN S HOSPITAL OF PITTSBURGH   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA 1 ANTITRYPSIN; CAENORHABDITIS ELEGANS PROTEIN; HYBRID PROTEIN; INSULIN; NHX-2 PROTEIN, C ELEGANS; SERINE PROTEINASE INHIBITOR; SODIUM PROTON EXCHANGE PROTEIN;

ALPHA 1 ANTITRYPSIN DEFICIENCY; ANIMAL; CAENORHABDITIS ELEGANS; CELL LINE; ENDOPLASMIC RETICULUM; ENDOPLASMIC RETICULUM ASSOCIATED DEGRADATION; GENE EXPRESSION; GENETICS; HUMAN; METABOLISM; MOUSE; MUTATION; PHENOTYPE; PROMOTER REGION; PROTEIN DEGRADATION; PROTEOSTASIS DEFICIENCY; REPORTER GENE; RNA INTERFERENCE; SIGNAL TRANSDUCTION; TRANSGENIC ANIMAL; TRANSGENIC MOUSE; ULTRASTRUCTURE;

ALPHA 1-ANTITRYPSIN; ALPHA 1-ANTITRYPSIN DEFICIENCY; ANIMALS; ANIMALS, GENETICALLY MODIFIED; CAENORHABDITIS ELEGANS; CAENORHABDITIS ELEGANS PROTEINS; CELL LINE; ENDOPLASMIC RETICULUM; ENDOPLASMIC RETICULUM-ASSOCIATED DEGRADATION; GENE EXPRESSION; GENES, REPORTER; HUMANS; INSULIN; MICE; MICE, TRANSGENIC; MUTATION; PHENOTYPE; PROMOTER REGIONS, GENETIC; PROTEOLYSIS; PROTEOSTASIS DEFICIENCIES; RECOMBINANT FUSION PROTEINS; RNA INTERFERENCE; SERPINS; SIGNAL TRANSDUCTION; SODIUM-HYDROGEN ANTIPORTER;

EID: 84929021779     PISSN: None     EISSN: 14602083     Source Type: Journal    
DOI: 10.1093/hmg/ddu235     Document Type: Article

References (0)