Large proteoglycan complexes and disturbed collagen architecture in the corneal extracellular matrix of mucopolysaccharidosis type VII (Sly syndrome).

@article{Young2011LargePC,
  title={Large proteoglycan complexes and disturbed collagen architecture in the corneal extracellular matrix of mucopolysaccharidosis type VII (Sly syndrome).},
  author={Robert D Young and Petra Liskova and Christian Pinali and Barbara Palka and Michalis Palos and Kateřina Jirsov{\'a} and Enkela Hrdlickova and Marketa Tesarova and Milan Elleder and Jiř{\'i} Zeman and Keith M. Meek and Carlo Knupp and Andrew J Quantock},
  journal={Investigative ophthalmology & visual science},
  year={2011},
  volume={52 9},
  pages={6720-8}
}
PURPOSE Deficiencies in enzymes involved in proteoglycan (PG) turnover underlie a number of rare mucopolysaccharidoses (MPS), investigations of which can considerably aid understanding of the roles of PGs in corneal matrix biology. Here, the authors analyze novel pathologic changes in MPS VII (Sly syndrome) to determine the nature of PG-collagen associations in stromal ultrastructure. METHODS Transmission electron microscopy and electron tomography were used to investigate PG-collagen… CONTINUE READING
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Genetic basis of corneal diseases and the role of keratocytes in corneal transparency: a review

  • GK Klintworth, JV Jester
  • Clin Exp Ophthalmol. 2010;38:23–33
  • 2011
1 Excerpt

Application of (lamellar) keratoplasty and limbal stem cell transplantation for corneal clouding in the mucopolysaccharidoses—a review

  • L Pinello, M Busin, L Fontana, HS. Dua
  • Clin Exp Ophthalmol
  • 2010
1 Excerpt

Gene therapy for ocular problems in mucopolysaccharidosis: an experimental and promising approach with benefits in animal models—a review

  • KP Ponder, A. Auricchio
  • Clin Exp Ophthalmol
  • 2010

Ocular manifestations in the mucopolysaccharidoses: a review

  • JL Ashworth, FE Kruse, B Bachmann
  • Clin Exp Ophthalmol
  • 2010

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