title: Microfibrillar protein and phospholipid in granular corneal dystrophy.
abstract: Keratoplasty specimens from eight patients with granular corneal dystrophy (GCD) and age-matched control subjects were examined by combinations of immunohistological stains, transmission electron microscopy (TEM), and sodium dodecyl sulfate gel electrophoresis. Fresh frozen sections from corneas with GCD stained positively with antibodies to microfibrillar protein by immunofluorescence. Routine TEM disclosed that the granules had central electron-dense areas partially surrounded by 9- to 10-nm tubular microfibrils. Material eluted from corneas with GCD showed denser peptide bands at 65 and 110 kilo than in normal corneas. Stains were negative for elastin, amyloid, neutral lipids, cholesterol, and glycosaminoglycan. Luxol fast blue MBSN stain was strongly positive in the granules in all cases examined. Immunofluorescent stains were negative with antibodies to plasma fibronectin (cold insoluble globulin), laminin, collagens I to V, basement membrane proteoglycan, tropoelastin, and keratin. In two corneas with GCD an increased lipid content was found in every phospholipid class, although cholesterol content was unchanged. Alterations in the fatty acid profiles of phospholipids were also observed.
mesh_terms: D003285:Contractile Proteins / Q000378:metabolism*; D003315:Cornea / Q000032:analysis / Q000648:ultrastructure; D003317:Corneal Dystrophies, Hereditary / Q000378:metabolism* / Q000473:pathology; D004591:Electrophoresis, Polyacrylamide Gel; D004848:Epithelium / Q000648:ultrastructure; D016326:Extracellular Matrix Proteins; D005455:Fluorescent Antibody Technique; D006801:Humans; D008854:Microscopy, Electron; D010743:Phospholipids / Q000378:metabolism*; D000072260:RNA Splicing Factors; D013194:Staining and Labeling
chemical_list: D003285:Contractile Proteins; D016326:Extracellular Matrix Proteins; D010743:Phospholipids; D000072260:RNA Splicing Factors; C016598:microfibrillar protein