4-phenylbutyrate attenuates the ER stress response and cyclic AMP accumulation in DYT1 dystonia cell models

PLoS ONE

Volumn 9, Issue 11, 2014, Pages

  Cho, Jin A ^a   Zhang, Xuan ^b   Miller, Gregory M ^c   Lencer, Wayne I ^a,d   Nery, Flavia C ^b  

^a BOSTON CHILDREN S HOSPITAL   (United States)

^b MASSACHUSETTS GENERAL HOSPITAL   (United States)

^c NORTHEASTERN UNIVERSITY   (United States)

^d HARVARD UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

4 PHENYLBUTYRIC ACID; CELL PROTEIN; CYCLIC AMP; FORSKOLIN; TORSINA PROTEIN; UNCLASSIFIED DRUG; 4-PHENYLBUTYRIC ACID; ARYLBUTYRIC ACID DERIVATIVE; CHAPERONE; TOR1A PROTEIN, HUMAN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BIOACCUMULATION; CELL DAMAGE; CELL LINE; CELL REGENERATION; CONTROLLED STUDY; DRUG MECHANISM; DYSTONIA; DYT1 DYSTONIA CELL; EMBRYO; ENDOPLASMIC RETICULUM STRESS; FEMALE; HUMAN; HUMAN CELL; MOUSE; MUSCLE CELL; MUTATION; NONHUMAN; PROTEIN DEFECT; PROTEIN FOLDING; SIGNAL TRANSDUCTION; ANIMAL; DOWN REGULATION; DRUG EFFECTS; DYSTONIA MUSCULORUM DEFORMANS; METABOLISM; PATHOLOGY;

ANIMALS; CELL LINE; CYCLIC AMP; DOWN-REGULATION; DYSTONIA MUSCULORUM DEFORMANS; ENDOPLASMIC RETICULUM STRESS; HUMANS; MICE; MOLECULAR CHAPERONES; PHENYLBUTYRATES; SIGNAL TRANSDUCTION;

EID: 84915745960     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0110086     Document Type: Article

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