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Neurocutaneous Syndromes

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Abstract

Tuberous sclerosis (TS) is a dominantly inherited disorder that affects multiple organ systems [1]–[3]. The current diagnostic criteria require TS complex-associated lesions of two or more organ systems or two dissimilar lesions of the same organ system. TS is sporadic in 60%–70% of cases. Two genes have been identified: TSC1 (hamartin) located at 9q34 and TSC2 (tuberin) located at 16p13.3 [4]. The genes have tumor-suppressing activity, but the pathogenesis of TS is not fully understood.

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7 Neurocutaneous Syndromes

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© 2007 Springer

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(2007). Neurocutaneous Syndromes. In: Pediatric Neuropathology. Springer, Tokyo. https://doi.org/10.1007/978-4-431-49898-8_7

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  • DOI: https://doi.org/10.1007/978-4-431-49898-8_7

  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-70246-7

  • Online ISBN: 978-4-431-49898-8

  • eBook Packages: MedicineMedicine (R0)

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