Novel cerebral lesions in the Eker rat model of tuberous sclerosis: cortical tuber and anaplastic ganglioglioma.
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.
Mizuguchi
M
M
Department of Pediatrics, Jichi Medical School, Tochigi, Japan.
Takashima
S
S
Yamanouchi
H
H
Nakazato
Y
Y
Mitani
H
H
Hino
O
O
D000818:Animals; D000906:Antibodies; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology*; D004195:Disease Models, Animal; D004805:Ependyma / Q000002:abnormalities; D018303:Ganglioglioma / Q000235:genetics / Q000473:pathology*; D006222:Hamartoma / Q000235:genetics / Q000473:pathology; D009044:Motor Cortex / Q000737:chemistry / Q000473:pathology*; D017072:Neostriatum / Q000002:abnormalities; D009714:Nucleus Accumbens / Q000002:abnormalities; D010641:Phenotype; D051381:Rats; D011922:Rats, Mutant Strains; D012097:Repressor Proteins / Q000032:analysis / Q000235:genetics* / Q000276:immunology; D014402:Tuberous Sclerosis / Q000235:genetics / Q000473:pathology*; D000077005:Tuberous Sclerosis Complex 2 Protein; D025521:Tumor Suppressor Proteins
D000906:Antibodies; D012097:Repressor Proteins; C000624653:TSC2 protein, human; C000624655:Tsc2 protein, rat; D000077005:Tuberous Sclerosis Complex 2 Protein; D025521:Tumor Suppressor Proteins