Chapter 9. Pathogenesis of TSC in the Brain

  1. Dr. David J. Kwiatkowski3,
  2. Dr. Vicky Holets Whittemore4 and
  3. Dr. Elizabeth A. Thiele5
  1. Peter B. Crino1,
  2. Rupal Mehta2 and
  3. Harry V. Vinters2

Published Online: 9 JUN 2010

DOI: 10.1002/9783527630073.ch9

Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics

Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics

How to Cite

Crino, P. B., Mehta, R. and Vinters, H. V. (2010) Pathogenesis of TSC in the Brain, in Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics (eds D. J. Kwiatkowski, V. H. Whittemore and E. A. Thiele), Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim, Germany. doi: 10.1002/9783527630073.ch9

Editor Information

  1. 3

    Brigham & Women's Hospital, Dana Farber Cancer Institute, Harvard Medical School, 1 Blackfan Circle, Boston, MA 02115, USA

  2. 4

    Tuberous Sclerosis Alliance, 801 Roeder Road, Silver Spring, MD 20910, USA

  3. 5

    Carol & James Herscot Center For TCS, Massachusetts General Hospital, Department of Neurology, 175 Cambridge Street, Boston, MA 02114, USA

Author Information

  1. 1

    University of Pennsylvania, PENN Epilepsy Center, Philadelphia, PA 19104, USA

  2. 2

    David Geffen School of Medicine at UCLA, Department of Pathology & Laboratory Medicine, Los Angeles, CA 90095, USA

Publication History

  1. Published Online: 9 JUN 2010
  2. Published Print: 21 APR 2010

ISBN Information

Print ISBN: 9783527322015

Online ISBN: 9783527630073

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Keywords:

  • giant cell;
  • SEGAs;
  • subependymal nodule;
  • tuber;
  • tuberous sclerosis complex;
  • ventricular zone

Summary

This chapter contains sections titled:

  • Introduction

  • Tubers

  • SENs and SEGAs

  • Cell Lineage

  • mTOR Activation and Biallelic TSC Gene Inactivation

  • Alternative Signaling Cascades in TSC Brain Lesions

  • Structural Alterations in Nontuber Brain Areas

  • Conclusions and Future Directions

  • References