X-ray diffraction and transmission electron microscopy of Morquio syndrome type A cornea: A structural analysis

Cornea

Volumn 16, Issue 3, 1997, Pages 369-376

  Rawe, I M ^a,b   Leonard, D W ^a   Meek, K M ^a   Zabel, R W ^a,c  

^a OPEN UNIVERSITY   (United Kingdom)

^b SCHEPENS EYE RESEARCH INSTITUTE   (United States)

^c Eye Associates   (Canada)

Author keywords

Cornea; Morquio syndrome; Mucopolysaccharidosis; Synchrotron; X ray diffraction

Indexed keywords

COLLAGEN; PROTEOGLYCAN;

ADULT; ARTICLE; CASE REPORT; CORNEA DISEASE; CORNEA STROMA; FEMALE; HUMAN; HUMAN TISSUE; MORQUIO SYNDROME; PENETRATING KERATOPLASTY; PRIORITY JOURNAL; STRUCTURE ANALYSIS; SYNCHROTRON; TRANSMISSION ELECTRON MICROSCOPY; ULTRASTRUCTURE; X RAY DIFFRACTION;

ADULT; COLLAGEN; CORNEA; CORNEAL DISEASES; CORNEAL STROMA; FEMALE; HUMANS; KERATOPLASTY, PENETRATING; MICROSCOPY, ELECTRON; MUCOPOLYSACCHARIDOSIS IV; PROTEOGLYCANS; SPECIMEN HANDLING; X-RAY DIFFRACTION;

EID: 0030994701     PISSN: 02773740     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (31)

1. Beck M, Glössl J, Grubisic A, Spranger J. Heterogeneity of Morquio disease. Clin Genet 1986;29:325-31.
2. Olsen H, Baggesen K, Sjolie AK. Cataracts in Morquio syndrome. Ophthalmol Pediatr Genet 1993;14:87-9.
3. Orii T, Kiman T, Sukegawa K, Kanemura T, Hattori S, Taga T, Ko K. Late onset N-acetylgalactosamine-6-sulfate sulfatase deficiency in two brothers. Connect Tissue Res 1981;13:169-75.
4. Glössl J, Maroteaux P, DiNatale P, Kresse H. Different properties of residual N-acetylgalactosamine-6-sulfate sulfatase in fibroblasts from patients with mild and severe forms of Morquio disease type A. Pediatr Res 1981;15:976-8.
5. Glössl J, Kresse H. Impaired degradation of keratan sulfate by Morquio A fibroblasts. Biochem J 1982;203:2335-8.
6. Iwamoto M, Nawa Y, Maumenee IH, Young-Ramsaran J, Matalon R, Green WR. Ocular histopathology and ultrastructure of Morquio syndrome (systemic mucopolysaccharidosis IV A). Graefes Arch Clin Exp Ophthalmol 1990;228:342-9.
7. Minami R, Katsuyuki A, Kudoh T, Tsugawa S, Oyanagi K, Nakao T. Identification of keratan sulfate in liver affected by Morquio syndrome. Clin Chim Acta 1979;93:207-13.
8. Hart RW, Farrell RA. Light scattering in the cornea. J Opt Soc Am 1969;59:766-74.
9. Benedek GB. The theory of transparency of the eye. Appl Opt 1971;10:459-73.
10. Fullwood NJ, Meek KM. An ultrastructural, time-resolved study of freezing in the corneal stroma. J Mol Biol 1994;236:749-58.
11. Meek KM, Elliott GF, Nave C. A synchrotron x-ray diffraction study of bovine cornea stained with cupromeronic blue. Coll Relat Res 1986;6:203-18.
12. Freund DE, McCally RL, Farrell RA, Cristol SM, L'Hernault NL, Edelhauser HF. Ultrastructure in anterior and posterior stroma of perfused human and rabbit corneas. Invest Ophthalmol Vis Sci 1995;36:1508-23.
13. Goodfellow JM, Elliott OF, Woolgar AE. X-ray diffraction studies of the corneal stroma. J Mol Biol 1978;119:237-52.
14. Meek KM, Leonard D. Ultrastructure of the corneal stroma: a comparative study. Biophys J 1993;64:273-80.
15. Meek KM, Elliott GF, Sayers Z, Whitburn SB, Koch MHJ. Interpretation of the meridional X-ray diffraction pattern from collagen fibrils in corneal stroma. J Mol Biol 1982;149:477-88.
16. Fullwood NJ, Tuft SJ, Malik NJ, Meek KM, Ridgway AEA, Harrison RJ. Synchrotron X-ray diffraction studies of keratoconus corneal stroma. Invest Ophthalmol Vis Sci 1992;33: 1734-41.
17. Meek KM, Fullwood NJ, Cooke PH, et al. Synchrotron x-ray diffraction studies of the cornea, with implications for stromal hydration. Biophys J 1991;60:467-74.
18. Scott JE. The electron histochemistry of proteoglycans in connective tissues. Inst Phys Conf Ser No 93 1988;3:405-9.
19. Rawe IM, Zabel RW, Tuft SJ, Chen V, Meek KM. A morphological study of rabbit corneas after laser keratectomy. Eye 1992;6:637-42.
20. Rawe IM, Tuft SJ, Meek KM. Proteoglycan and collagen morphology in superficially scarred rabbit cornea. Histochem J 1992;24:311-8.
21. Kanai A, Kaufman H. Electron microscopic studies of corneal stroma: age changes of collagen fibres. Ann Ophthalmol 1973;5:285-92.
22. Maroudas A, Wachtel E, Grushko G, Katz EP, Weinberg P. The effect of osmotic and mechanical pressures on water partitioning in articular cartilage. Biochim Biophys Acta 1991;1073:285-94.
23. Quantock AJ, Meek KM, Fullwood NJ, Zabel RW. Scheie's syndrome: the architecture of corneal collagen and distribution of corneal proteoglycans. Can J Ophthalmol 1993;28: 266-72.
24. Huang Y, Bron AJ, Meek KM, Vellodi A, McDonald B. Ultrastructural study of the cornea in a bone marrow-transplanted Hurler syndrome patient. Exp Eye Res 1996;62:377-87.
25. Marshall GE, Konstas AGP, Lee WR. Collagen in ocular tissues. Br J Ophthalmol 1993;77:515-24.
26. Chapman JA, Armitage PM. An analysis of fibrous long spacing forms of collagen. Connect Tissue Res 1972;1:31-7.
27. Scott JE, Parry DAD. Control of collagen fibril diameters in tissues. Int J Biol Macromol 1992;14:292-3.
28. Leonard DW. The ultrastructure of the corneal stroma and its implications for transparency. PhD thesis, 1995. The Open University, Milton Keynes.
29. Leonard DW, Meek KM. Estimation of the refractive indices of the collagen fibrils and ground substance of the corneal stroma using data from x-ray diffraction. Biophys J (in press).
30. Rosen DA, Haust MD, Yamashita T, Bryans MD. Keratoplasty and electron microscopy of the cornea in systemic mucopolysaccharidosis (Hurler's disease). Can J Ophthalmol 1968;3:218-30.
31. Constantopoulos GG, Scott JA, Shull RM. Corneal opacity in canine MPS1: changes after bone marrow transplantation. Invest Ophthalmol Vis Sci 1989;30:1802-7.