Crystallographic Structure of Human β-Hexosaminidase A: Interpretation of Tay-Sachs Mutations and Loss of GM2 Ganglioside Hydrolysis

Journal of Molecular Biology

Volumn 359, Issue 4, 2006, Pages 913-929

  Lemieux, M Joanne ^a   Mark, Brian L ^a   Cherney, Maia M ^a   Withers, Stephen G ^b   Mahuran, Don J ^c   James, Michael N G ^a  

^a UNIVERSITY OF ALBERTA   (Canada)

^b UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^c HOSPITAL FOR SICK CHILDREN   (Canada)

Author keywords

glycoside hydrolase; GM2 ganglioside; lysosomal storage disorders; Tay Sachs disease; hexoasaminidase A

Indexed keywords

BETA N ACETYLHEXOSAMINIDASE A; CARBOXYLIC ACID DERIVATIVE; GANGLIOSIDE GM2; GANGLIOSIDE GM2 ACTIVATOR PROTEIN; HETERODIMER; N ACETYLGLUCOSAMINE THIAZOLINE; N ACETYLNEURAMINIC ACID; SUICIDE SUBSTRATE; UNCLASSIFIED DRUG;

ARTICLE; CRYSTAL STRUCTURE; LIPID HYDROLYSIS; MUTATION; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN FOLDING; PROTEIN FUNCTION; PROTEIN STRUCTURE; TAY SACHS DISEASE; X RAY CRYSTALLOGRAPHY;

EID: 33744798282     PISSN: 00222836     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jmb.2006.04.004     Document Type: Article

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