Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking α-l-iduronidase.

@article{Holley2011MucopolysaccharidosisTI,
  title={Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking α-l-iduronidase.},
  author={Rebecca J. Holley and Audrey Deligny and Wei Wei and H. Angharad Watson and Milady Ni{\~n}onuevo and Anders Dag{\"a}lv and Julie A. Leary and Brian W Bigger and Lena Kjell{\'e}n and Catherine L. R. Merry},
  journal={The Journal of biological chemistry},
  year={2011},
  volume={286 43},
  pages={
          37515-24
        }
}
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-iduronidase (Idua) deficiency. HS levels were significantly increased in liver and brain tissue from 12-week-old Idua(-/-) mice by 87- and 20-fold, respectively. In addition, HS chains were shown to contain… CONTINUE READING
BETA

Similar Papers

Citations

Publications citing this paper.
SHOWING 1-10 OF 24 CITATIONS

Heparan Sulfate Inhibits Hematopoietic Stem and Progenitor Cell Migration and Engraftment in Mucopolysaccharidosis I*

  • The Journal of biological chemistry
  • 2014
VIEW 5 EXCERPTS
CITES METHODS, RESULTS & BACKGROUND
HIGHLY INFLUENCED

Mucopolysaccharidosis type I: current knowledge on its pathophysiological mechanisms

  • Metabolic Brain Disease
  • 2012
VIEW 7 EXCERPTS
CITES BACKGROUND & RESULTS
HIGHLY INFLUENCED

Neuropathology in Mouse Models of Mucopolysaccharidosis Type I, IIIA and IIIB

VIEW 5 EXCERPTS
CITES BACKGROUND, METHODS & RESULTS
HIGHLY INFLUENCED

The role of innate immunity in mucopolysaccharide diseases

  • Journal of neurochemistry
  • 2018
VIEW 6 EXCERPTS
CITES BACKGROUND
HIGHLY INFLUENCED