Cardiovascular Pathology
Volume 20, Issue 5 , Pages 315-321 , September 2011

Clear cells in the atrioventricular valves of infants with severe human mucopolysaccharidosis (Hurler syndrome) are activated valvular interstitial cells

  • Elizabeth Braunlin

      Affiliations

    • Department of Pediatrics, University of Minnesota Hospital/Fairview, Minneapolis, MN, USA
    • Corresponding Author InformationCorresponding author. Department of Pediatrics, University of Minnesota Hospital/Fairview, MMC 94, Minneapolis, MN 55455, USA. Tel.: +1 612 626 2755; fax: +1 612 626 2467.
    • ,
  • Jakub Tolar

      Affiliations

    • Department of Pediatrics, University of Minnesota Hospital/Fairview, Minneapolis, MN, USA
    • ,
  • Shannon Mackey-Bojack

      Affiliations

    • The Jesse E. Edwards Registry of Cardiovascular Disease, St. Paul, MN, USA
    • ,
  • Tiwanda Masinde

      Affiliations

    • Cellular and Molecular Pathology Branch, National Institute of Environmental Health Sciences, Research Triangle Park, NC, USA
    • ,
  • William Krivit

      Affiliations

    • Department of Pediatrics, University of Minnesota Hospital/Fairview, Minneapolis, MN, USA
    • Deceased.
    • ,
  • Frederick J. Schoen

      Affiliations

    • Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA

Received 18 February 2010 ,Revised 6 May 2010 ,Accepted 2 June 2010.

References 

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  2. Bach G, Friedman R, Weissmann B, Neufeld EF. The defect in the Hurler and Scheie syndromes: deficiency of α-l-iduronidase. Proc Natl Acad Sci USA. 1972;69:2048–2051
  3. Renteria VG, Ferrans VJ, Roberts WC. The heart in the Hurler syndrome. Gross, histologic and ultrastructural observations in five necropsy cases. Am J Cardiol. 1976;38:487–501
  4. Braunlin EA, Stauffer NR, Peters CH, Berry JM, Bass JL, Hopwood JJ, et al. Usefulness of bone marrow transplantation in the Hurler syndrome. Am J Cardiol. 2003;92:882–886
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  14. Ginsberg SD, Galvin JE, Lee VM, Rorke LB, Dickson DW, Wolfe JH, et al. Accumulation of intracellular amyloid-β-peptide (Aβ1-40) in mucopolysaccharidosis brains. J Neuropathol Exp Neurol. 1999;58(8):815–824
  15. Latif N, Sarathchandra P, Taylor PM, Antoniw J, Yacoub MH. Localization and pattern of expression of extracellular matrix components in human heart valves. J Heart Valve Dis. 2005;14(2):218–227
  16. Grande-Allen KJ, Calabro A, Gupta V, Wight TN, Hascall VC, Vesely I. Glycosaminoglycans and proteoglycans in normal mitral valve leaflets and chordae: association with regions of tensile and compressive loading. Glycobiology. 2004;14(7):621–633
  17. Gupta V, Barzilla JE, Mendez JS, Stephens EH, Lee EL, Collard CD, et al. Abundance and location of proteoglycans and hyaluronan within normal and myxomatous mitral valves. Cardiovasc Pathol. 2009;18(4):191–197
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  19. Schoen FJ. Evolving concepts of cardiac valve dynamics: the continuum of development, function structure, pathobiology, and tissue engineering. Circulation. 2008;118(18):1864–1880

 This work was supported by a grant from the Vikings Fund at the University of Minnesota Medical Foundation.

 Elizabeth Braunlin, MD, PhD receives speaker’s honoraria from Genzyme and Biomarin companies, both of which produce an enzyme treatment for MPS I.

 Jakub Tolar, MD, PhD receives speaker’s honoraria from Genzyme company.

PII: S1054-8807(10)00076-1

doi: 10.1016/j.carpath.2010.06.004

Cardiovascular Pathology
Volume 20, Issue 5 , Pages 315-321 , September 2011

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