Abstract
The Eker rat is a model for human tuberous sclerosis (TSC) caused by a mutation in the Tsc2 gene. We describe here histological and immunohistochemical findings of the brain lesions in Eker rats, with emphasis on 2 novel lesions found in this study: a cortical tuber and an anaplastic ganglioglioma. The rat cortical tuber resembled those of humans, and further confirmed the value of this animal model as a tool for investigating the molecular pathology of tuberous sclerosis. On the other hand, the rat anaplastic ganglioglioma had features of a malignant neoplasm that are absent from human subependymal giant cell astrocytomas.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Antibodies
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Brain Neoplasms / genetics
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Brain Neoplasms / pathology*
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Disease Models, Animal*
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Ependyma / abnormalities
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Ganglioglioma / genetics
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Ganglioglioma / pathology*
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Hamartoma / genetics
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Hamartoma / pathology
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Motor Cortex / chemistry
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Motor Cortex / pathology*
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Neostriatum / abnormalities
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Nucleus Accumbens / abnormalities
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Phenotype
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Rats
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Rats, Mutant Strains*
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Repressor Proteins / analysis
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Repressor Proteins / genetics*
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Repressor Proteins / immunology
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Tuberous Sclerosis / genetics
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Tuberous Sclerosis / pathology*
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Tuberous Sclerosis Complex 2 Protein
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Tumor Suppressor Proteins
Substances
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Antibodies
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Repressor Proteins
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TSC2 protein, human
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Tsc2 protein, rat
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Tuberous Sclerosis Complex 2 Protein
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Tumor Suppressor Proteins