Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.36 (hyaluronidase)
 4,606 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Keratoplasty specimens from 19 patients with macular corneal dystrophy (MCD), 11 patients with lattice corneal dystrophy (LCD) and 2 patients with granular corneal dystrophy (GCD) were examined by combinations of histochemistry, electron microscopy and electron--histochemistry. Electron histochemistry disclosed that the deposits of MCD have sulfate chondroitin and another hyaluronidase--resistant glycoaminoglycan and that the deposits of LCD have a little sulfate chondroitin. The authors suggest: (1) the possible pathologic mechanism of MCD is that the keratocytes and endothelial cells synthesize abnormal fibrillogranular material which consists of glycoaminoglycan, glycoprotein and lipid; (2) LCD is a primary localized corneal amyloidosis in which the amyloid deposits may result from corneal epithelial cells and keratocytes with a little sulfate chondroitin; (3) the deposits synthesized by corneal epithelial cells and keratocytes in GCD may result from a genetic defect in processing or synthesizing proteins.
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PMID:[Macular, lattice and granular dystrophy of the cornea: ultra-histochemistry and ultrastructure study]. 251 54

We describe a family with an unusual autosomal dominant anterior corneal dystrophy. The onset was characterized by frequent, recurrent corneal erosions in the first decade. This subsided during adolescence and was followed by progressive decreased vision. Slit-lamp examination revealed bilateral subepithelial opacities and haze, involving the entire cornea, but most dense centrally. Histopathological study revealed a subepithelial band of eosinophilic, periodic acid-Schiff-positive, alcian blue-positive, hyaluronidase-sensitive material anterior to Bowman's layer. Electron microscopy demonstrated subepithelial deposition of fine fibrillar material consistent with glycosaminoglycan. Immunohistochemical analysis indicated that the accumulated material contained a combination of chondroitin 4-sulfate and dermatan sulfate. This unique condition clinically resembled Grayson-Wilbrandt dystrophy, but differed histochemically. To our knowledge, this anterior corneal dystrophy has not previously been reported, and it is best described by the name "subepithelial mucinous corneal dystrophy."
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PMID:Subepithelial mucinous corneal dystrophy. Clinical and pathological correlations. 835 93