title: Neuronal and glial properties of a murine transgenic retinoblastoma model.
abstract: Antigenic properties of a murine transgenic model for hereditary retinoblastoma, induced by a chimeric gene coding for Simian virus 40 large T antigen, an oncogene that inactivates the retinoblastoma susceptibility gene product, were studied by immunohistochemistry. All transgenic mice develop bilateral intraocular retinal tumors in the inner nuclear layer with Homer Wright-like rosettes, and one quarter develop midbrain tumors resembling trilateral retinoblastoma. Cell lines TE-1 and TM-1 were established from intraocular and metastatic tumors, respectively. Intraocular tumors reacted with antibodies to neuron-specific enolase and synaptophysin, while vimentin, glial fibrillary acidic, and S-100 proteins were detected only in reactive glia derived from adjacent retina. The midbrain tumors showed weak reactivity to synaptophysin, and they blended with reactive astrocytes positive for glial markers. The tumors were negative for cytokeratins. Finally both derived cell lines expressed synaptophysin and individual neurofilament triplet proteins in immunofluorescence and Western blotting, supporting their essentially neuronal nature. The antigenic profile resembles human retinoblastoma, but differences in morphology and antigen distribution suggest a more close relationship to neurons of the inner nuclear layer than to photoreceptor cells.
authors.affiliation: Department of Ophthalmology, University of Helsinki, Finland.
authors.forename: T
authors.initials: T
authors.lastname: Kivelä
authors.forename: I
authors.initials: I
authors.lastname: Virtanen
authors.forename: D M
authors.initials: DM
authors.lastname: Marcus
authors.forename: J M
authors.initials: JM
authors.lastname: O'Brien
authors.forename: J L
authors.initials: JL
authors.lastname: Carpenter
authors.forename: E
authors.initials: E
authors.lastname: Brauner
authors.forename: A
authors.initials: A
authors.lastname: Tarkkanen
authors.forename: D M
authors.initials: DM
authors.lastname: Albert
mesh_terms: D000818:Animals; D001344:Autopsy; D015153:Blotting, Western; D004195:Disease Models, Animal; D005134:Eye Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D005455:Fluorescent Antibody Technique; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D007150:Immunohistochemistry; D007381:Intermediate Filament Proteins / Q000378:metabolism; D007633:Keratins / Q000378:metabolism; D008565:Membrane Proteins / Q000378:metabolism; D051379:Mice; D008822:Mice, Transgenic / Q000235:genetics*; D016900:Neurofilament Proteins; D009457:Neuroglia / Q000378:metabolism / Q000473:pathology*; D009474:Neurons / Q000378:metabolism / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D012160:Retina / Q000378:metabolism / Q000473:pathology; D012175:Retinoblastoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D016708:Synaptophysin; D014407:Tumor Cells, Cultured / Q000378:metabolism / Q000473:pathology; D014746:Vimentin / Q000378:metabolism
chemical_list: D005904:Glial Fibrillary Acidic Protein; D007381:Intermediate Filament Proteins; D008565:Membrane Proteins; D016900:Neurofilament Proteins; D016708:Synaptophysin; D014746:Vimentin; D007633:Keratins; D010751:Phosphopyruvate Hydratase