Ultrastructural aspects of corneal fibrous tissue in the Scheie syndrome.
An ultrastructural study of the corneal fibrous tissue was performed in a case of Scheie's syndrome. Mucopolysaccharidosis deposits in keratocytes were observed as electron-clear and electron-dense inclusions. Modifications of the extracellular space included modifications of lamellar collagen organization and local hypertrophy of collagen bundles; presence of microfibrillar dense material isolating large irregular collagen fibers; and presence of fibrous long spacing type collagen fibers. The significance of these changes is discussed. This special form of collagen organization is supposed to appear in a modified microenvironment, that is the presence of an abnormal concentration of proteoglycans.
D001706:Biopsy; D003094:Collagen; D003315:Cornea / Q000473:pathology / Q000648:ultrastructure*; D005110:Extracellular Space / Q000648:ultrastructure; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D008854:Microscopy, Electron; D009083:Mucopolysaccharidoses / Q000473:pathology*; D008059:Mucopolysaccharidosis I / Q000473:pathology*; D011509:Proteoglycans
D011509:Proteoglycans; D003094:Collagen