Journal
BRAIN & DEVELOPMENT
Volume 23, Issue 7, Pages 508-515Publisher
ELSEVIER
DOI: 10.1016/S0387-7604(01)00304-7
Keywords
cortical tuber; Eker rat; hamartin; immunohistochemistry; subependymal giant cell astrocytoma; tuberin
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In the cerebrum of patients with tuberous sclerosis (TSC), there are three types of nodular lesions: cortical tubers, subcortical heterotopic nodules and subependymal giant cell astrocytomas. Histologically, these hamartias and hamartomas contain abnormal giant cells that show evidence of abnormal differentiation of immature neural cells. Recent identification of the TSC1 and TSC2 genes has facilitated studies of the molecular pathology of TSC. The expression of their protein products, hamartin and tuberin, is altered in various TSC lesions. However, the molecular mechanism by which cortical tubers develop remains to be elucidated. The Eker rat, a naturally occurring animal model of TSC, will provide a powerful tool for future investigations of TSC. (C) 2001 Elsevier Science B.V. All rights reserved.
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