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Publication : Primitive neuroectodermal tumor of the midbrain in a murine model of retinoblastoma.

First Author  Marcus DM Year  1991
Journal  Invest Ophthalmol Vis Sci Volume  32
Issue  2 Pages  293-301
PubMed ID  1993580 Mgi Jnum  J:103617
Mgi Id  MGI:3610524 Citation  Marcus DM, et al. (1991) Primitive neuroectodermal tumor of the midbrain in a murine model of retinoblastoma. Invest Ophthalmol Vis Sci 32(2):293-301
abstractText  The first heritable model of retinoblastoma was established by retina-specific expression of simian virus 40 T-antigen (SV40 T-ag) in transgenic mice. Bilateral, multifocal ocular tumors were observed in 100% of transgene-bearing mice. Central nervous system neoplasms occurred at a lower rate (27%) and represented the murine counterpart of human trilateral retinoblastoma. The authors characterized the transgenic brain tumors and found them to be primitive neuroectodermal tumors (PNET) of the midbrain. Murine brain tumors do not involve the pineal gland and most closely resemble undifferentiated suprasellar or parasellar tumors occasionally observed in human trilateral retinoblastoma. The murine malignancies arose from the subependymal cells of the cerebral aqueduct. Immunohistochemical and ultrastructural examination revealed that the transgenic brain tumors were undifferentiated and lacked all antigens associated with normal murine neuronal, glial, and ependymal cells.
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