Activation of the Unfolded Protein Response in Sporadic Inclusion-Body Myositis but Not in Hereditary GNE Inclusion-Body Myopathy

Journal of Neuropathology and Experimental Neurology

Volumn 74, Issue 6, 2015, Pages 538-546

  Nogalska, Anna ^a   D'Agostino, Carla ^a   Engel, W King ^a   Cacciottolo, Mafalda ^a   Asada, Shinichi ^b,c   Mori, Kazutoshi ^b   Askanas, Valerie ^a  

^a UNIVERSITY OF SOUTHERN CALIFORNIA   (United States)

^b KYOTO UNIVERSITY   (Japan)

^c NIIGATA UNIVERSITY OF PHARMACY AND APPLIED LIFE SCIENCES   (Japan)

Author keywords

Cultured human muscle fibers; Endoplasmic reticulum stress; GNE; Hereditary inclusion body myopathy; Sporadic inclusion body myositis; UDP N acetylglucosamine 2 epimerase N acetylmannosamine kinase; Unfolded protein response

Indexed keywords

ACTIVATING TRANSCRIPTION FACTOR 4; GLUCOSE REGULATED PROTEIN 78; X BOX BINDING PROTEIN 1; ACTIVATING TRANSCRIPTION FACTOR 6; ATF4 PROTEIN, HUMAN; ATF6 PROTEIN, HUMAN; CADHERIN; DNA BINDING PROTEIN; ENZYME INHIBITOR; HEAT SHOCK PROTEIN; MESSENGER RNA; MOLECULAR CHAPERONE GRP78; MULTIENZYME COMPLEX; REGULATORY FACTOR X TRANSCRIPTION FACTORS; TRANSCRIPTION FACTOR; UDP-N-ACETYLGLUCOSAMINE 2-EPIMERASE - N-ACETYLMANNOSAMINE KINASE;

ADULT; AGED; ARTICLE; CLINICAL ARTICLE; CONTROLLED STUDY; ENDOPLASMIC RETICULUM STRESS; FEMALE; GENE MUTATION; HUMAN; HUMAN CELL; HUMAN TISSUE; INCLUSION BODY MYOSITIS; MALE; MUSCLE BIOPSY; MUSCLE CELL; PRIORITY JOURNAL; UNFOLDED PROTEIN RESPONSE; CELL CULTURE; DISTAL MYOPATHY; DRUG EFFECTS; GENETICS; METABOLISM; MIDDLE AGED; MUTATION; PATHOLOGY; PATHOPHYSIOLOGY; PHYSIOLOGY; SKELETAL MUSCLE;

ACTIVATING TRANSCRIPTION FACTOR 4; ACTIVATING TRANSCRIPTION FACTOR 6; AGED; CADHERINS; CELLS, CULTURED; DISTAL MYOPATHIES; DNA-BINDING PROTEINS; ENZYME INHIBITORS; FEMALE; HEAT-SHOCK PROTEINS; HUMANS; MALE; MIDDLE AGED; MULTIENZYME COMPLEXES; MUSCLE FIBERS, SKELETAL; MUSCLE, SKELETAL; MUTATION; MYOSITIS, INCLUSION BODY; RNA, MESSENGER; TRANSCRIPTION FACTORS; UNFOLDED PROTEIN RESPONSE;

EID: 84942878803     PISSN: 00223069     EISSN: 15546578     Source Type: Journal    
DOI: 10.1097/NEN.0000000000000196     Document Type: Article

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