Mucopolysaccharidosis Type I, Unique Structure of Accumulated Heparan Sulfate and Increased N-Sulfotransferase Activity in Mice Lacking α-l-iduronidase

Mucopolysaccharide (MPS) diseases are characterized by accumulation of glycosaminoglycans (GAGs) due to deficiencies in lysosomal enzymes responsible for GAG breakdown. Using a murine model of MPSI Hurler (MPSIH), we have quantified the heparan sulfate (HS) accumulation resulting from α-l-iduronidas...

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Détails bibliographiques
Auteurs principaux: Holley, Rebecca J., Deligny, Audrey, Wei, Wei, Watson, H. Angharad, Niñonuevo, Milady R., Dagälv, Anders, Leary, Julie A., Bigger, Brian W., Kjellén, Lena, Merry, Catherine L. R.
Format: Artigo
Langue:Inglês
Publié: American Society for Biochemistry and Molecular Biology 2011
Sujets:
Glycobiology and Extracellular Matrices
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC3199497/
https://ncbi.nlm.nih.gov/pubmed/21873421
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M111.287474
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