Proteoglycans contain a 4.6 A repeat in muscular dystrophy corneas: x-ray diffraction evidence
| Autor: | M. S. Capel, David J. Schanzlin, Andrew J. Quantock, E. J.-M. A. Thonar, G. K. Klintworth, M. E. Lenz |
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| Rok vydání: | 1996 |
| Předmět: |
Macular corneal dystrophy
Adult Male Keratan sulfate Lumican Macromolecular Substances Biophysics Biophysical Phenomena Amidohydrolases Glycosaminoglycan Cornea chemistry.chemical_compound X-Ray Diffraction medicine Humans Peptide-N4-(N-acetyl-beta-glucosaminyl) Asparagine Amidase Muscular dystrophy Aged Glycosaminoglycans Corneal Dystrophies Hereditary Chondroitin Lyases nutritional and metabolic diseases medicine.disease Molecular biology eye diseases medicine.anatomical_structure Biochemistry chemistry Keratan Sulfate X-ray crystallography Ultrastructure Female Proteoglycans sense organs Collagen Research Article |
| Zdroj: | Biophysical Journal. 70(4):1966-1972 |
| ISSN: | 0006-3495 |
| DOI: | 10.1016/s0006-3495(96)79761-8 |
| Popis: | Synchrotron x-ray diffraction patterns from macular corneal dystrophy (MCD) corneas contain an unusual reflection that arises because of an undefined ultrastructure with a periodic repeat in the region of 4.6 A. In this study, we compared with wide-angle x-ray diffraction patterns obtained from four normal human corneas and four MCD corneas. Moreover, portions of two of the MCD corneas were pretreated with a specific glycosidase to shed light on the origin of the 4.6 A reflection. None of the normal corneas produced an x-ray reflection in the region of 4.6 A, whereas all four of the MCD corneas did (MCD type I at 4.65 A and 4.63 A, MCD type II at 4.63 A and 4.67 A). This reflection was diminished after incubation of the MCD tissues with either chondroitinase ABC or N-glycanase. The findings indicate that glycosaminoglycans or proteoglycans contribute to the unusual MCD x-ray reflection and hence most likely contain a periodic 4.6 A ultrastructure. Furthermore, the results imply that periodic 4.6 A MCD ultrastructures reside in either intact, unsulfated lumican molecules and regions of the CS/DS-containing molecules or in a region of a hybrid macromolecular aggregate formed by the interaction of the two molecules. |
| Databáze: | OpenAIRE |
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