13. Other Neurological Diseases

  1. Robert Crichton and
  2. Roberta Ward

Published Online: 6 SEP 2013

DOI: 10.1002/9781118553480.ch13

Metal-based Neurodegeneration: From Molecular Mechanisms to Therapeutic Strategies

Metal-based Neurodegeneration: From Molecular Mechanisms to Therapeutic Strategies

How to Cite

Crichton, R. and Ward, R. (eds) (2013) Other Neurological Diseases, in Metal-based Neurodegeneration: From Molecular Mechanisms to Therapeutic Strategies, John Wiley and Sons Ltd, Chichester, United Kingdom. doi: 10.1002/9781118553480.ch13

Editor Information

  1. Unit of Biochemistry, Université Catholique de Louvain, Belgium

Publication History

  1. Published Online: 6 SEP 2013
  2. Published Print: 25 OCT 2013

ISBN Information

Print ISBN: 9781119977148

Online ISBN: 9781118553480

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Keywords:

  • aceruloplasminaemia;
  • neurodegeneration with brain iron accumulation (NBIA);
  • neuroferritinopathy;
  • neurological diseases;
  • Wilson's and Menkes diseases

Summary

This chapter explains two classic disorders of copper (Cu) metabolism, Wilson's disease and Menkes disease, both caused by defective copper transporting ATPases. Next, it discusses another Cu-linked neurodegenerative disease, aceruloplasminaemia, which does not affect Cu metabolism, but rather has major effects on iron metabolism. Aceruloplasminaemia is one of a number of neurodegenerative disorders associated with brain iron accumulation (NBIA) which encompasses a wide spectrum of both adult and paediatric neurological diseases. These include neuroferritinopathy caused by a mutation in the ferritin light chain; and pantothenate kinase-associated neurodegeneration (PKAN, due to mutations in the PANK2 gene), formerly known as Hallervorden—Spatz syndrome. The chapter concludes with a brief description of multiple sclerosis, an autoimmune disease resulting in demyelination in the central nervous system (CNS), and HIV-associated dementia, a progressive encephalopathy observed in AIDS patients.