Ultrastructural study of the cornea in a bone marrow-transplanted Hurler syndrome patient

Experimental Eye Research

Volumn 62, Issue 4, 1996, Pages 377-388

  Huang, Y ^a   Bron, A J ^b   Meek, K M ^a   Vellodi, A ^c   McDonald, B ^b  

^a OPEN UNIVERSITY   (United Kingdom)

^b UNIVERSITY OF OXFORD   (United Kingdom)

^c UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

Bone marrow transplantation; Cornea; Histochemistry; Hurler syndrome; Long spacing collagen; Mucopolysaccharidosis; Proteoglycans; Ultrastructure; X ray diffraction

Indexed keywords

ADOLESCENT; ARTICLE; BONE MARROW TRANSPLANTATION; CASE REPORT; CORNEA OPACITY; FEMALE; HUMAN; HUMAN TISSUE; HURLER SYNDROME; MUCOPOLYSACCHARIDOSIS; PRIORITY JOURNAL; ULTRASTRUCTURE;

EID: 0029982078     PISSN: 00144835     EISSN: None     Source Type: Journal    
DOI: 10.1006/exer.1996.0043     Document Type: Article

References (38)

1. Bajart, A. M. and Pavilack, M. A. (1994). Corneal manifestations of metabolic disease. In Principles and Practice of Ophthalmology: Clinical Practice. (Eds Albert, D. M. et al.). P. 294. W. B. Saunders: San Diego, U.S.A.
2. Craig, A. S. and Parry, D. A. D. (1981). Collagen fibrils of the vertebrate corneal stroma. J. Ultrastruct. Mol. Struct. Res. 74, 232-9.
3. Dohlman, C. H. (1960). Metabolism of the corneal graft. In The Transparency of the Cornea. Symposium: The Council for International Organizations of Medical Sciences (UNESCO and WHO). P. 182. Charles, C. Thomas, Springfield, U.S.A.
4. Fullwood, N. J., Tuft, S. J., Malik, N. S., Meek, K.M., Ridgway, A. E. A. and Harrison, R. J. (1992). Synchrotron X-ray diffraction studies of keratoconus corneal stroma. Invest. Ophthalmol. Vis. Sci. 33, 1734-41.
5. Fratzl, P. and Daxer, A. (1993). Structural transformation of collagen fibrils in corneal stroma during drying, an x-ray scattering study. Biophys. J. 64, 1210-1214.
6. Gills, P. J., Hobson, R., Hanley, W. B. and McKusick, V. A. (1965). Electroretinography and fundus oculi findings in Hurler's disease and allied mucopolysaccharidosis. Arch. Ophthalmol. 74, 596-603.
7. Hart, R. W. and Farrell, FR. A. (1969). Light scattering in the cornea. J. Opt. Sco. Am. 59, 766-74.
8. Hirano, K., Awaya, S., Kobayashi, M., et al. (1988). Ultrastructural and quantitative studies on age-related changes in the extracellular matrices of the human trabecular meshwork. Nihon Ganka Kiyo (Folia Ophthalmol Jpn) 39, 1680-8.
9. Hirano, K., Nakamura, M., Kobayashi, M., Kobayashi, K., Hoshino, T. and Awaya, S. (1993). Long-spacing collagen in the human corneal stroma. Jpn J. Ophthalmol. 37, 148-55.
10. Hoogerbrugge, P. M., Vellodi, A. and Pasquini, R. (1995). Bone marrow transplantation for inborn errors of metabolism. In Colour Atlas and Text of Bone Marrow Transplantation. (Eds Treleaven, J. and Wiernik, P.). Pp. 19-35. Mosby-Wolfe, London.
11. Kenyon, K. R., Quigley, H. A., Hussels, J. E. and Wyllie, R. G. (1972). The systemic mucopolysaccharidoses: ultrastructural and histochemical studies of conjunctiva and skin. Am. J. Ophthalmol. 73, 811-33.
12. Lüllmann-Rauch, R. (1990). Mucopolysaccharidosis (MPS) in ocular tissues as induced by amphiphilic di-cationic drugs. Lens Eye Toxicity Res. 7, 263-79.
13. Lütjen-Drecoll, E., Rittig, M., Rauterberg, J., Jander, R. and Mollenhauer, J. (1989). Immunomicroscopical study of type VI collagen in the trabecular meshwork of normal and glaucomatous eyes. Exp. Eye Res. 48, 139-47.
14. Marshall, G. E., Kontas, A. G. and Lee, W. R. (1991). Immunogold fine structural localization of extracellular matrix components in aged human cornea II. Collagen types V and VI. Graefe's Arch Clin. Exp. Ophthalmol. 229, 164-71.
15. Matsuda, H., Satake, Y. and Katsumata, H. (1970). Ultrastructural observations on the cornea of Hurler syndrome. Acta Sco. Ophth. Jap. 74, 47.
16. Maurice, D. M. (1957). The structure and transparency of the cornea. J. Physiol. 136, 263-86.
17. Mckusick, V. A., Kaplan, D., Wise, D., Hanley, W. B., Suddarth, S. B., Sevick, M. E. and Maumenee, A. E. (1965). The genetic mucopolysaccharidoses. Medecine 44, 445.
18. McMenamin, P. G., Lee, W. R. and Aitken, D. A. N. (1986). Age-related changes in the human outflow apparatus. Ophthalmology 93, 194-209.
19. Meek, K. M., Blamires, T., Elliott, G. F., Gyi, T. J. and Nave, C. (1987). The organisation of collagen fibrils in the human corneal stroma: a synchrotron x-ray diffraction study. Curr. Eye Res. 6, 841-6.
20. Meek, K. M., Quantock, A. J., Elliott, G. F., Ridgway, A. E., Tullo, A. B., Bron, A. J. and Thonar, E. J. (1989). Macular corneal dystrophy: the macromolecular structure of the stroma observed using electron microscopy and synchrotron X-ray diffraction. Exp. Eye Res. 49, 941-58.
21. Meek, K. M., Fullwood, N. J., Cooke, P. H., Elliott, G. F., Maurice, D. M., Quantock, A. J., Wall, R. S. and Worthington, C. R. (1991). Synchrotron x-ray diffraction studies of the cornea, with implications for stromal hydration. Biophys. J 60, 467-74.
22. Meek, K. M. and Leonard, D. W. (1993). Ultrastructure of the corneal stroma: a comparative study. Biophys. J. 64, 273-80.
23. Neufeld, E. F. and Muenzer, J. (1989). Mucopolysaccharidosis. In The Metabolic Basis of Inherited Disease. (Eds. Scriver, C. R., Beaudet, A. L., Sly, W. S. and Valle, D.) 6th ed, Pp. 1565-1587. McGraw-Hill: New York, U.S.A.
24. Quantock, A. J., Meek, K. M., Fullwood, N. J. and Zabel, R. W. (1993). Scheie's syndrome: the architecture of corneal collagen and distribution of corneal proteoglycans. Can. J. Ophthalmol. 28, 266-72.
25. Rosen, D. A., Haust, M. D., Yamashita, T. and Bryans, A. M. (1968). Keratoplasty and electron microscopy of the cornea in systemic mucopolysaccharidosis (Hurler's disease). Can. J Ophthalmol. 3, 218.
26. Scheie, H. G., Hambrick, G. W. and Barness, L. A. (1962). A newly recognized forme fruste of Hurler's disease (Gargoylism). Am. J. Ophthal. 53, 753.
27. Schwarz, M. F., Werblin, T. P. and Green, W. R. (1985). Occurrence of mucopolysaccharide in corneal grafts in the Maroteaux-Lamy syndrome. Cornea. 4, 58-66.
28. Scott, J. E., Orford, R. and Hughes, E. W. (1981). Proteoglycan-collagen arrangements in developing rat tail tendon. Biochem J. 195, 573-81.
29. Scott, J. E. and Haigh, M. (1985). "Small"-proteoglycan: collagen interactions: keratan sulphate proteoglycan associates with rabbit corneal collagen fibrils at the "a" and "c" bands. Biosci. Rep. 5, 765-74.
30. Scott, J. E. (1988). Proteoglycan-fibrillar collagen interactions. Biochem. J. 252, 313-23.
31. Spellacy, E., Bankes, K. J. L., Crow, J., Doumashkin, R., Shah, D. and Watts, R. W. (1980). Glaucoma in a case of Hurler disease. Br. J. Ophthalmol. 64, 773.
32. Summers, C. G., Purple, R. L., Krivit, W., Pineda, R., Copland, G. T., Ramsay, N. K., Kersey, J. H. and Whitley, C. B. (1989). Ocular changes in the mucopolysaccharidosis after bone marrow transplantation. A preliminary report. Ophthalmology 96, 977-84.
33. Takahashi, T., Cho, H. I., Kubblin, C. L. and Cintron, C. (1993). Keratan sulfate and dermatan sulfate proteoglycans associate with type VI collagen in fetal rabbit cornea. J. Histochem. Cytochem. 10, 1447-57.
34. Tuft, S. J., Gartry, D. S., Rawe, I. M. and Meek, K. M. (1993). Photorefractive keratectomy: implications of corneal wound healing. Br. J. Ophthalmol. 77, 243-7.
35. Vanshtein, B. K. (1966). Diffraction of X-rays by Chain Molecules. Elsevier: Amsterdam.
36. Whitley, C. B., Belani, K. G., Chang, P. N., Summers, C. G., Blazer, B. R., Tsai, M. Y., Latchaw, R. E., Ramsay, N. K. and Kersey, J. H. (1993). Long-term outcome of Hurler syndrome following bone marrow transplantation. Am. J. Med. Genet. 46, 209-18.
37. Worthington, C. R. and Inouye, H. (1985). X-ray diffraction study of the cornea. Int. J. Biol. Macromol. 7, 2-8.
38. Zimmerman, D. R., Trüeb, B., Winterhalter, K. H., Witmer, R. and Fischer, R. W. (1986). Type VI collagen is a major component of the human cornea. FEBS Lett. 197, 55-8.