Biochemical characterization of the Cys138 Arg substitution associated with the AB variant form of G(M2) gangliosidosis: Evidence that Cys138 is required for the recognition of the G(M2) activator/G(M2) ganglioside complex by β-hexosaminidase A

Biochemistry

Volumn 37, Issue 3, 1998, Pages 814-821

  Xie, Bei ^a,b   Rigat, Brigitte ^a   Smiljanic Georgijev, Natasha ^a,b   Deng, Huinan ^a   Mahuran, Don ^a,b  

^a HOSPITAL FOR SICK CHILDREN RESEARCH INSTITUTE   (Canada)

^b UNIVERSITY OF TORONTO   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

BETA N ACETYLHEXOSAMINIDASE A; GANGLIOSIDE GM2; ARGININE; BETA N ACETYLHEXOSAMINIDASE; CYSTEINE; DISULFIDE; FLAG PEPTIDE; GANGLIOSIDE GM2 ACTIVATOR PROTEIN; HYBRID PROTEIN; PEPTIDE; PROTEIN;

AMINO ACID SUBSTITUTION; ANIMAL CELL; ARTICLE; CHO CELL; CIRCULAR DICHROISM; CONTROLLED STUDY; ENZYME CHEMISTRY; ENZYME SPECIFICITY; ENZYME SUBSTRATE COMPLEX; ESCHERICHIA COLI; GM2 GANGLIOSIDOSIS; HYDROLYSIS; MOLECULAR INTERACTION; NONHUMAN; PRIORITY JOURNAL; STRUCTURE ACTIVITY RELATION; AMINO ACID SEQUENCE; ANIMAL; BIOSYNTHESIS; ENZYMOLOGY; GANGLIOSIDOSIS; GENETIC VARIABILITY; GENETICS; HAMSTER; METABOLISM; MOLECULAR GENETICS; PHYSIOLOGY; PROTEIN FOLDING;

AMINO ACID SEQUENCE; AMINO ACID SUBSTITUTION; ANIMALS; ARGININE; BETA-N-ACETYLHEXOSAMINIDASE; CHO CELLS; CRICETINAE; CYSTEINE; DISULFIDES; ESCHERICHIA COLI; G(M2) ACTIVATOR PROTEIN; G(M2) GANGLIOSIDE; GANGLIOSIDOSES; MOLECULAR SEQUENCE DATA; PEPTIDES; PROTEIN FOLDING; PROTEINS; RECOMBINANT FUSION PROTEINS; VARIATION (GENETICS);

EID: 0032548279     PISSN: 00062960     EISSN: None     Source Type: Journal    
DOI: 10.1021/bi971211s     Document Type: Article

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