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Macular corneal dystrophy

C-GlcNAc6ST Human 1 Macular corneal dystrophy type 1, II Loss of vision 50... [Pg.319]

Hassell JR, Newsome DA, Krachmer JH, Rodrigues MM. Macular corneal dystrophy failure to synthesize a mature keratan sulfate proteoglycan. Proc Natl Acad Sci U S A 1980 77 3705-3709. [Pg.173]

Macular corneal dystrophy is a rare dystrophy that is characterized by abnormal deposits in the corneal stroma, keratocytes, Descemet s membrane, and... [Pg.181]

Figure 1 Schematic diagram of the eye in horizontal section indicating each ocular component, a, corneal epithelium b, keratocyte c, corneal endothelium d, aqueous humor e, conjunctiva /, sclera g, trabecular meshwork h, iris i, lens /, ciliary zonule and body k, vitreous /, retina m, interphotoreceptor matrix n, retinal pigment epithelium o, Bruch s membrane p, choroid q, optic nerve head r, lamina cribrosa extraocular muscles and tissues. The candidate glycosaminoglycans involved in the ocular components of each eye disease described in this chapter are as follows macular corneal dystrophy (b, c KS, CS/DS, HA), glaucoma (d HA g CS/DS, HS, HA q, r CS, HS, HA), cataract r. CS/DS, HS, HA), diabetic retinopathy (fc HA / HS), retinal detach-ment/proliferative vitreoretinopathy k, I, m, n CS/DS, HS, HA), myopia (f, p CS), thyroid eye disease (s CS, HA), pseudoexfoliation syndrome (c, d, g, h, i, j KS, CS/DS, HA). KS, keratan sulfate CS/DS, chondroitin sulfate/dermatan sulfate HS, heparan sulfate HA, hyaluronan. Figure 1 Schematic diagram of the eye in horizontal section indicating each ocular component, a, corneal epithelium b, keratocyte c, corneal endothelium d, aqueous humor e, conjunctiva /, sclera g, trabecular meshwork h, iris i, lens /, ciliary zonule and body k, vitreous /, retina m, interphotoreceptor matrix n, retinal pigment epithelium o, Bruch s membrane p, choroid q, optic nerve head r, lamina cribrosa extraocular muscles and tissues. The candidate glycosaminoglycans involved in the ocular components of each eye disease described in this chapter are as follows macular corneal dystrophy (b, c KS, CS/DS, HA), glaucoma (d HA g CS/DS, HS, HA q, r CS, HS, HA), cataract r. CS/DS, HS, HA), diabetic retinopathy (fc HA / HS), retinal detach-ment/proliferative vitreoretinopathy k, I, m, n CS/DS, HS, HA), myopia (f, p CS), thyroid eye disease (s CS, HA), pseudoexfoliation syndrome (c, d, g, h, i, j KS, CS/DS, HA). KS, keratan sulfate CS/DS, chondroitin sulfate/dermatan sulfate HS, heparan sulfate HA, hyaluronan.
Macular corneal dystrophy can be classified into three immunophenotypes, types I, lA, and II, according to the serum level of sulfated keratan sulfate and immunoreactivity of the corneal tissue. In macular corneal dystrophy type I, the keratan sulfate level is low in both the serum and corneal tissue. In macular... [Pg.182]

CS/DS Macular corneal dystrophy, glaucoma, cataract, retinal detachment/proliferative vitreoretinopathy, myopia, thyroid eye disease, pseudoexfoliation syndrome HS Glaucoma, cataract, diabetic retinopathy, retinal detachment/proliferative... [Pg.183]

HA Macular corneal dystrophy, glaucoma, cataract, diabetic retinopathy, retinal... [Pg.183]

The keratan and chondroitin/dermatan sulfate levels in normal human corneas and corneas affected by macular corneal dystrophies types I and II were compared (10). The results revealed that the keratan sulfate chain size was reduced and chain sulfation was absent in type I, and that sulfation of both GlcNAc and Gal was significantly reduced in type II. The chondroitin/dermatan sulfate chain sizes were also decreased in all diseased corneas, and the contents of 4- and... [Pg.183]

Macular corneal dystrophy types I and II have also been characterized histo-chemically. In normal corneas, high levels of sulfated keratan sulfate were detected in the stroma. Bowman s layer, and Descemet s membrane with low levels in the keratocytes, epithelium, and endothelium. Furthermore, in normal corneas, negligible levels of labeling for A-acetyllactosamine (unsulfated keratan sulfate) were detected. In macular corneal dystrophy type I corneas, sulfated keratan sulfate... [Pg.183]

Klintworth GK, Smith CF. Abnormalities of proteoglycans and glycoproteins synthesized by corneal organ cultures derived from patients with macular corneal dystrophy. Lab Invest 1983 48 603-612. [Pg.199]

Nakazawa K, Hassell JR, Hascall VC, Lohmander LS, Newsome DA, Krachmer J. Defective processing of keratan sulfate in macular corneal dystrophy. J Biol Chem 1984 259 13751-13757. [Pg.199]

Hasegawa N, Torii T, Kato T, Miyajima H, Furuhata A, Nakayasu K, Kanai A, Habuchi O. Decreased GlcNAc 6-O-sulfotransferase activity in the cornea with macular corneal dystrophy. Invest Ophthalmol Vis Sci 2000 41 3670-3677. [Pg.199]

Akama TO, Nishida K, Nakayama J, Watanabe H, Ozaki K, Nakamura T, Dota A, Kawasaki S, Inoue Y, Maeda N, Yamamoto S, Fujiwara T, Thonar EJ, Shimomura Y, Kinoshita S, Tanigami A, Fukuda MN. Macular corneal dystrophy... [Pg.199]

El-Ashry MF, Abd El-Aziz MM, Wilkins S, Cheetham ME, Wilkie SE, Hard-castle AJ, Halford S, Bayoumi AY, Ficker LA, Tuft S, Bhattacharya SS, Ebenezer ND. Identification of novel mutations in the carbohydrate sulfo-transferase gene (CHST6) causing macular corneal dystrophy. Invest Ophthalmol Vis Sci 2002 43 377-382. [Pg.200]

Niel F, Ellies P, Dighiero P, Soria J, Sabbagh C, San C, Renard G, Delpech M, Valleix S. Truncating mutations in the carbohydrate sulfotransferase 6 gene (CHST6) result in macular corneal dystrophy. Invest Ophthalmol Vis Sci 2003 44 2949-2953. [Pg.200]

Lewis D, Davies Y, Nieduszynski lA, Lawrence F, Quantock AJ, Bonshek R, Fullwood NJ. Ultrastructural localization of sulfated and unsulfated keratan sulfate in normal and macular corneal dystrophy type I. Glycobiology 2000 10 305-312. [Pg.200]

Ouantock AJ, Fullwood NJ, Thonar EJ, Waltman SR, Capel MS, Ito M, Verity SM, Schanzlin DJ. Macular corneal dystrophy type II multiple studies on a cornea with low levels of sulphated keratan sulphate. Eye 1997 11 57-67. [Pg.200]

See other pages where Macular corneal dystrophy is mentioned: [Pg.2288]    [Pg.323]    [Pg.343]    [Pg.181]    [Pg.181]    [Pg.182]    [Pg.183]    [Pg.183]    [Pg.184]    [Pg.200]    [Pg.5]    [Pg.1817]    [Pg.2299]   
See also in sourсe #XX -- [ Pg.181 , Pg.182 , Pg.183 ]

See also in sourсe #XX -- [ Pg.719 ]



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