The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse

Molecular Genetics and Metabolism

Volumn 105, Issue 1, 2012, Pages 116-125

  Wang, Dan ^a   Belakhov, Valery ^b   Kandasamy, Jeyakumar ^b   Baasov, Timor ^b   Li, Su Chen ^c   Li, Yu Teh ^c   Bedwell, David M ^a,d   Keeling, Kim M ^d  

^a UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^b TECHNION ISRAEL INSTITUTE OF TECHNOLOGY   (Israel)

^c TULANE UNIVERSITY   (United States)

^d University of Alabama at Birmingham   (United States)

Author keywords

Aminoglycosides; Hurler syndrome; MPS I H; Nonsense mutations; Readthrough; Suppression

Indexed keywords

AMIKACIN; AMINOGLYCOSIDE DERIVATIVE; AMINOGLYCOSIDE NB54; AMINOGLYCOSIDE NB84; GENTAMICIN; GLYCOSAMINOGLYCAN; LEVO IDURONIDASE; PAROMOMYCIN; PROTEIN; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; EMBRYO; FIBROBLAST; HUMAN; HUMAN CELL; MOUSE; NONHUMAN; NONSENSE MUTATION; PRIORITY JOURNAL;

AMINOGLYCOSIDES; ANIMALS; BASE SEQUENCE; BIOLOGICAL ASSAY; CODON, NONSENSE; DESIGNER DRUGS; DISEASE MODELS, ANIMAL; EMBRYO, MAMMALIAN; FIBROBLASTS; GENES, REPORTER; GLYCOSAMINOGLYCANS; IDURONIDASE; LYSOSOMES; MICE; MICE, INBRED C57BL; MOLECULAR SEQUENCE DATA; MUCOPOLYSACCHARIDOSIS I; TRISACCHARIDES;

MUS;

EID: 84855345879     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2011.10.005     Document Type: Article

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