A genetic model of substrate reduction therapy for mucopolysaccharidosis

Journal of Biological Chemistry

Volumn 287, Issue 43, 2012, Pages 36283-36290

  Lamanna, William C ^a   Lawrence, Roger ^a   Sarrazin, Stéphane ^a,c   Lameda Diaz, Carlos ^a   Gordts, Philip L S M ^a   Moremen, Kelley W ^b   Esko, Jeffrey D ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b UNIVERSITY OF GEORGIA   (United States)

^c UNIVERSITÉ DE LYON   (France)

Author keywords

[No Author keywords available]

Indexed keywords

BIOSYNTHETIC ENZYMES; CHAIN ASSEMBLIES; ENZYME REPLACEMENT THERAPY; GENETIC MODELS; GLYCOSAMINOGLYCANS; HEPARAN SULFATES; HETEROZYGOSITY; LYSOSOMAL STORAGE DISORDER; PROOF OF PRINCIPLES;

ANIMAL CELL CULTURE; BIOCHEMISTRY; BIOSYNTHESIS; DISEASE CONTROL; DISEASES; GENE THERAPY; MAMMALS; PATHOLOGY; SUBSTRATES; SULFUR COMPOUNDS; TISSUE;

ENZYME INHIBITION;

GLUCOSAMINE DERIVATIVE; GLYCOSAMINOGLYCAN; HEPARAN SULFATE; LYSOSOME ENZYME; SULFAMIDASE; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; EMBRYO; ENZYME METABOLISM; ENZYME PURIFICATION; ENZYME REPLACEMENT; ENZYME SYNTHESIS; EXT1 GENE; EXT2 GENE; FIBROBLAST; GENE; GENE SILENCING; GENETIC MODEL; HETEROZYGOSITY; HUMAN; HUMAN CELL; IMMUNOHISTOCHEMISTRY; LYSOSOME; METHODOLOGY; MOUSE; MUCOPOLYSACCHARIDOSIS; NONHUMAN; PRIORITY JOURNAL; SANFILIPPO SYNDROME TYPE A; SUBSTRATE REDUCTION THERAPY;

ANIMALS; HEPARITIN SULFATE; HUMANS; MICE; MICE, KNOCKOUT; MODELS, GENETIC; MUCOPOLYSACCHARIDOSES; N-ACETYLGLUCOSAMINYLTRANSFERASES;

EID: 84867826038     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M112.403360     Document Type: Article

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