title: Descemet's membrane in the iridocorneal-endothelial syndrome: morphology and composition.
abstract: The iridocorneal-endothelial syndrome is a disease of the ocular anterior segment characterized by corneal failure, glaucoma and iris destruction. Specular photomicroscopical and histological studies suggest the disorder is caused by a population of abnormal corneal endothelial cells. In other corneal endotheliopathies Descemet's membrane, the basement membrane underlying the endothelial cells, is disfigured by the presence of an abnormal region of extracellular matrix termed a posterior collagenous layer, which is laid down by the diseased endothelial cells. In this study we sought to establish the typical morphology and composition of Descemet's membrane in the iridocorneal-endothelial syndrome. Ultrastructural examination of Descemet's membrane in 27 keratoplasty specimens identified three morphologic patterns. In the majority there was a posterior collagenous layer which in all cases consisted of an anterior layer of wide-spaced collagen and a posterior layer of microfibrils embedded in an amorphous matrix. In four specimens which did not possess a posterior collagenous layer the anterior banded zone of Descemet's membrane was absent. In five corneas Descemet's membrane was normal. The composition of the posterior collagenous layer was examined by immunoelectron microscopy (five corneas) and histochemistry (six corneas). Collagen Types I, III, V, VI and VIII, fibronectin, tenascin and oxytalan were microfibrillar components, collagen Type VIII formed wide-spaced collagen whilst laminin was present in the amorphous matrix. The stereotyped derangements of structure and composition identified in the endothelial basement membrane may significantly influence the pathobiology of this disorder.
authors.affiliation: Department of Histopathology, Charing Cross and Westminster Medical School, London, U.K.
authors.forename: S G
authors.initials: SG
authors.lastname: Levy
authors.forename: A C
authors.initials: AC
authors.lastname: McCartney
authors.forename: H
authors.initials: H
authors.lastname: Sawada
authors.forename: P J
authors.initials: PJ
authors.lastname: Dopping-Hepenstal
authors.forename: R A
authors.initials: RA
authors.lastname: Alexander
authors.forename: J
authors.initials: J
authors.lastname: Moss
mesh_terms: D000328:Adult; D001284:Atrophy; D001485:Basement Membrane / Q000648:ultrastructure; D003094:Collagen / Q000648:ultrastructure; D003285:Contractile Proteins / Q000648:ultrastructure; D003316:Corneal Diseases / Q000473:pathology; D003886:Descemet Membrane / Q000648:ultrastructure*; D004547:Elastic Tissue / Q000648:ultrastructure; D004728:Endothelium, Corneal / Q000473:pathology*; D016326:Extracellular Matrix Proteins; D005353:Fibronectins / Q000648:ultrastructure; D005901:Glaucoma / Q000473:pathology*; D006801:Humans; D007498:Iris / Q000473:pathology*; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D000072260:RNA Splicing Factors; D013577:Syndrome; D019063:Tenascin / Q000648:ultrastructure
chemical_list: D003285:Contractile Proteins; D016326:Extracellular Matrix Proteins; D005353:Fibronectins; D000072260:RNA Splicing Factors; D019063:Tenascin; C016598:microfibrillar protein; D003094:Collagen