4-Phenylbutyrate rescues trafficking incompetent mutant α-galactosidase A without restoring its functionality

Biochemical and Biophysical Research Communications

Volumn 360, Issue 2, 2007, Pages 375-380

  Yam, Gary Hin Fai ^a   Roth, Jürgen ^a   Zuber, Christian ^a  

^a UNIVERSITY OF ZURICH   (Switzerland)

Author keywords

Galactosidase A; Chaperone; Endoplasmic reticulum; Fabry disease; Lysosomes; Protein quality control

Indexed keywords

4 PHENYLBUTYRIC ACID; ALPHA GALACTOSIDASE; CHAPERONE;

ANIMAL CELL; ARTICLE; CONTROLLED STUDY; ENDOPLASMIC RETICULUM; ENZYME RELEASE; FABRY DISEASE; FIBROBLAST; HUMAN; HUMAN CELL; LYSOSOME; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN FUNCTION; PROTEIN QUALITY; TRANSGENIC MOUSE;

ALPHA-GALACTOSIDASE; ANIMALS; CELLS, CULTURED; DOSE-RESPONSE RELATIONSHIP, DRUG; FABRY DISEASE; FIBROBLASTS; HUMANS; MICE; MICE, TRANSGENIC; MUTATION; PHENYLBUTYRATES; PROTEIN TRANSPORT;

MUS MUSCULUS;

EID: 34447103301     PISSN: 0006291X     EISSN: 10902104     Source Type: Journal    
DOI: 10.1016/j.bbrc.2007.06.048     Document Type: Article

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