A thermodynamic assay to test pharmacological chaperones for Fabry disease

Biochimica et Biophysica Acta - General Subjects

Volumn 1840, Issue 3, 2014, Pages 1214-1224

  Andreotti, Giuseppina ^a   Citro, Valentina ^b   Correra, Antonella ^b,c   Cubellis, Maria Vittoria ^c,d  

^a CNR   (Italy)

^b INSTITUTE OF GENETICS AND BIOPHYSICS ADRIANO BUZZATI TRAVERSO   (Italy)

^c UNIVERSITY OF NAPLES FEDERICO II   (Italy)

^d CNR   (Italy)

Author keywords

Cell lysate; Limited proteolysis; Lysosomal storage disorder; Pharmacological chaperone; Urea induced unfolding

Indexed keywords

AGALSIDASE BETA; ALPHA GALACTOSIDASE; CALCIUM PHOSPHATE; CHAPERONE; LIPOFECTAMINE; MIGALASTAT; MUTANT PROTEIN; PHARMACOLOGICAL CHAPERONE; THERMOLYSIN; UNCLASSIFIED DRUG; UREA;

ANIMAL CELL; ARTICLE; CIRCULAR DICHROISM; CONTROLLED STUDY; EMBRYO; ENZYME ACTIVITY; ENZYME ASSAY; ENZYME DENATURATION; ENZYME STABILITY; FABRY DISEASE; HUMAN; HUMAN CELL; NONHUMAN; PH; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN UNFOLDING; THERMODYNAMICS; WESTERN BLOTTING; WILD TYPE;

1-DEOXY-GALACTONOJIRIMYCIN; AGAL; CD; CELL LYSATE; CIRCULAR DICHROISM; DGJ; FABRY DISEASE; FD; LIMITED PROTEOLYSIS; LYSOSOMAL ALPHA-GALACTOSIDASE; LYSOSOMAL STORAGE DISORDER; PC; PHARMACOLOGICAL CHAPERONE; PHARMACOLOGICAL CHAPERONES; UREA-INDUCED UNFOLDING;

ALPHA-GALACTOSIDASE; ANIMALS; CERCOPITHECUS AETHIOPS; COS CELLS; FABRY DISEASE; HEK293 CELLS; HUMANS; MOLECULAR CHAPERONES; MUTATION; PROTEIN FOLDING; PROTEIN STABILITY; THERMODYNAMICS; UREA;

EID: 84891868024     PISSN: 03044165     EISSN: 18728006     Source Type: Journal    
DOI: 10.1016/j.bbagen.2013.12.018     Document Type: Article

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