Chapter 6. Friedreich's Ataxia and Diseases Associated with Expansion of Non-Coding Triplets

  1. Robert R. Crichton and
  2. Roberta J. Ward

Published Online: 17 MAY 2006

DOI: 10.1002/0470022574.ch6

Metal-based Neurodegeneration: From Molecular Mechanisms to Therapeutic Strategies

Metal-based Neurodegeneration: From Molecular Mechanisms to Therapeutic Strategies

How to Cite

Crichton, R. R. and Ward, R. J. (2005) Friedreich's Ataxia and Diseases Associated with Expansion of Non-Coding Triplets, in Metal-based Neurodegeneration: From Molecular Mechanisms to Therapeutic Strategies, John Wiley & Sons, Ltd, Chichester, UK. doi: 10.1002/0470022574.ch6

Author Information

  1. Université Catholique de Louvain, Belgium

Publication History

  1. Published Online: 17 MAY 2006
  2. Published Print: 16 DEC 2005

ISBN Information

Print ISBN: 9780470022559

Online ISBN: 9780470022573

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Keywords:

  • cerebellar ataxia;
  • hypertrophic cardiomyopathy;
  • embryonic lethality;
  • iron-sulphur cluster biosynthesis;
  • Friedreich's ataxia;
  • triplet repeat expansions;
  • mitochondrial protein;
  • oxidative stress;
  • frataxin in iron metabolism

Summary

This chapter contains sections titled:

  • Incidence and pathophysiology of Friedreich's ataxia

  • Molecular basis of the disease – triplet repeat expansions

  • Molecular basis of the disease – frataxin and its role in iron metabolism

  • Other diseases associated with expansion of non-coding triplets

  • References