title: An immunohistochemical and electron microscopic study of extra-renal basement membranes in dogs with Samoyed hereditary glomerulopathy.
abstract: Samoyed hereditary glomerulopathy (SHG) in dogs has been employed as a model for human hereditary nephritis (HN), since affected dogs and patients show splitting of glomerular capillary basement membranes by electron microscopy (EM) and absent staining of glomerular capillaries for Goodpasture antigen (GPA) by immunofluorescence (IF). EM and IF were used to examine basement membranes (BM) in skin, lung, choroid plexus, lens, retina, and inner ear in SHG. By EM, BM in these tissues appeared similar in affected male, carrier female, and unaffected dogs. By IF, GPA could be detected only in lens capsule, internal limiting membrane of retina and basilar membrane of inner ear of unaffected and carrier female dogs, but not in affected male dogs. However, eye abnormalities and hearing loss were not present in any dogs, in contrast to their frequent occurrence in human HN. Our findings on extra-renal BM in SHG suggest that GPA is not required to maintain normal vision or hearing in affected male dogs and permit a greater understanding of the pathogenesis of human HN.
mesh_terms: D000818:Animals; D019867:Anti-Glomerular Basement Membrane Disease / Q000276:immunology*; D001324:Autoantigens / Q000032:analysis*; D001485:Basement Membrane / Q000276:immunology*; D004195:Disease Models, Animal; D004285:Dogs; D007758:Ear, Inner / Q000648:ultrastructure; D005260:Female; D005455:Fluorescent Antibody Technique; D006580:Genetic Carrier Screening; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology / Q000648:ultrastructure; D007908:Lens, Crystalline / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D009394:Nephritis, Hereditary / Q000473:pathology*; D009928:Organ Specificity; D012016:Reference Values; D012160:Retina / Q000648:ultrastructure
chemical_list: D001324:Autoantigens