{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T22:35:40Z","timestamp":1789598140969,"version":"build-2803163510"},"reference-count":73,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2007,2,13]],"date-time":"2007-02-13T00:00:00Z","timestamp":1171324800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Congenital Anomalies"],"published-print":{"date-parts":[[2007,3]]},"abstract":"<jats:p><jats:bold>ABSTRACT\u2003<\/jats:bold>Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutations of either of the two tumor suppressor genes,<jats:italic>TSC1<\/jats:italic>and<jats:italic>TSC2<\/jats:italic>, encoding hamartin and tuberin, respectively. TSC is pathologically characterized by the occurrence of multiple hamartias (focal dysplasias) and hamartomas (benign tumors) in the brain and many other organs. Cortical tubers are hamartias in the cerebral cortex responsible for many neuropsychiatric symptoms of TSC. Unlike TSC\u2010associated hamartomas, cortical tubers do not result from second somatic mutations of the<jats:italic>TSC<\/jats:italic>gene, and the mechanism by which they occur remains obscure. Histologically, the most conspicuous feature of cortical tubers is the presence of abnormal giant cells, which show abnormal size and differentiation. Recent studies on human TSC and its animal models have elucidated the critical roles of hamartin and tuberin regulating the growth and differentiation of neural cells.<\/jats:p>","DOI":"10.1111\/j.1741-4520.2006.00134.x","type":"journal-article","created":{"date-parts":[[2007,2,13]],"date-time":"2007-02-13T23:51:19Z","timestamp":1171410679000},"page":"2-8","source":"Crossref","is-referenced-by-count":24,"title":["Abnormal giant cells in the cerebral lesions of tuberous sclerosis 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