Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability

Biochemistry

Volumn 48, Issue 22, 2009, Pages 4816-4827

  Lieberman, Raquel L ^a,b,c   D'Aquino, J Alejandro ^b   Ringe, Dagmar ^b   Petsko, Gregory A ^a,b  

^a HARVARD MEDICAL SCHOOL   (United States)

^b BRANDEIS UNIVERSITY   (United States)

^c GEORGIA INSTITUTE OF TECHNOLOGY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACTIVE SITE; CONFORMATIONAL CHANGE; CONTROL REQUIREMENTS; ENDOPLASMIC RETICULUM; FABRY DISEASE; GALACTOSIDASE; GLOBOTRIAOSYLCERAMIDE; GLUCOSIDASE; GLYCOSIDASE; IMINOSUGAR; IMINOSUGARS; ISOFAGOMINE; LIGAND BINDING; LIPID METABOLISMS; LYSOSOMAL ENZYMES; LYSOSOMAL STORAGE DISORDER; MUTANT ENZYMES; PH SENSITIVE; SMALL MOLECULES; SPHINGOLIPIDS; THERMODYNAMIC PARAMETER; TWO-STATE; VAN'T HOFF;

ACIDS; BINDING SITES; BIOCHEMISTRY; CATALYSTS; CONFORMATIONS; DIFFERENTIAL SCANNING CALORIMETRY; DISEASE CONTROL; ENZYMES; QUALITY ASSURANCE; QUALITY CONTROL; QUALITY FUNCTION DEPLOYMENT; STRATEGIC PLANNING; TOTAL QUALITY MANAGEMENT;

PH EFFECTS;

1 DEOXYGALACTONOJIRIMYCIN; ALPHA GALACTOSIDASE; BETA GLUCOSIDASE; CHAPERONE; IMINOSUGAR; ISOFAGOMINE; LYSOSOME ENZYME; UNCLASSIFIED DRUG;

ARTICLE; CONFORMATIONAL TRANSITION; DIFFERENTIAL SCANNING CALORIMETRY; ENDOPLASMIC RETICULUM; ENZYME ACTIVITY; ENZYME STABILITY; ENZYME STRUCTURE; FABRY DISEASE; GAUCHER DISEASE; HUMAN; LIGAND BINDING; LYSOSOME; PH; PRIORITY JOURNAL; THERMOSTABILITY;

ALPHA-GALACTOSIDASE; BETA-GLUCOSIDASE; CRYSTALLOGRAPHY, X-RAY; DRUG DELIVERY SYSTEMS; ENZYME ACTIVATION; ENZYME STABILITY; HUMANS; HYDROGEN-ION CONCENTRATION; IMINO PYRANOSES; LYSOSOMES; MOLECULAR CHAPERONES; PROTEIN BINDING; RECOMBINANT PROTEINS; THERMODYNAMICS;

EID: 66649137718     PISSN: 00062960     EISSN: None     Source Type: Journal    
DOI: 10.1021/bi9002265     Document Type: Article

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