Chapter 17. Copper–Zinc Superoxide Dismutase, Its Copper Chaperone, and Familial Amyotrophic Lateral Sclerosis

  1. Marina Ramirez-Alvarado4,
  2. Jeffery W. Kelly5,
  3. Christopher M. Dobson6
  1. Duane D. Winkler1,
  2. Mercedes Prudencio2,
  3. Celeste Karch3,
  4. David R. Borchelt2,
  5. John Hart1

Published Online: 2 JUL 2010

DOI: 10.1002/9780470572702.ch17

Book Title

Protein Misfolding Diseases: Current and Emerging Principles and Therapies

Protein Misfolding Diseases: Current and Emerging Principles and Therapies

Additional Information

How to Cite

Winkler, D. D., Prudencio, M., Karch, C., Borchelt, D. R. and Hart, J. (2010) Copper–Zinc Superoxide Dismutase, Its Copper Chaperone, and Familial Amyotrophic Lateral Sclerosis, in Protein Misfolding Diseases: Current and Emerging Principles and Therapies (eds M. Ramirez-Alvarado, J. W. Kelly and C. M. Dobson), John Wiley & Sons, Inc., Hoboken, NJ, USA. doi: 10.1002/9780470572702.ch17

Editor Information

  1. 4

    Mayo Clinic College of Medicine, USA

  2. 5

    The Scripps Research Institute, USA

  3. 6

    University of Cambridge, UK

Author Information

  1. 1

    Department of Biochemistry and the X-ray Crystallography Core Laboratory, University of Texas Health Science Center at San Antonio, San Antonio, Texas, USA

  2. 2

    Department of Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, Florida, USA

  3. 3

    Department of Psychiatry, Washington University School of Medicine, St. Louis, Missouri, USA

Publication History

  1. Published Online: 2 JUL 2010
  2. Published Print: 9 JUN 2010

ISBN Information

Print ISBN: 9780471799283

Online ISBN: 9780470572702

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Keywords:

  • copper–zinc superoxide dismutase, its copper chaperone and familial amyotrophic lateral sclerosis;
  • copper chaperone for SOD1 and SOD1 maturation;
  • immature pathogenic SOD1 and toxicity

Summary

This chapter contains sections titled:

  • Structural Properties of Copper-Zinc Superoxide Dismutase

  • Genetics and Models of SOD1-Linked fALS

  • Aggregation of Mutant SOD1 in fALS

  • Copper Chaperone for SOD1 and SOD1 Maturation

  • Alternate Model for CCS Action

  • Immature Pathogenic SOD1 and Toxicity

  • Therapeutics

  • Conclusions

  • References

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