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Related Experiment Videos

The copper chaperone for superoxide dismutase

V C Culotta1, L W Klomp, J Strain

  • 1Division of Toxicological Sciences, Department of Environmental Health Sciences, Johns Hopkins University School of Public Health, Baltimore, Maryland 21205, USA. vculotta@phnet.sph.jhu.edu

The Journal of Biological Chemistry
|September 20, 1997
PubMed
Summary

Copper delivery to superoxide dismutase (SOD1) relies on a specific chaperone, LYS7/CCS. This chaperone is crucial for SOD1

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Area of Science:

  • Cellular Biology
  • Biochemistry
  • Neuroscience

Background:

  • Copper is essential for cellular function and is transported via specific pathways.
  • Copper/zinc superoxide dismutase (SOD1) requires copper for its enzymatic activity.
  • Dysfunctional SOD1 is implicated in neurodegenerative diseases like amyotrophic lateral sclerosis.

Purpose of the Study:

  • To identify the cellular factor responsible for copper delivery to SOD1.
  • To investigate the specificity of this copper delivery pathway.
  • To explore the therapeutic potential of understanding SOD1 copper delivery.

Main Methods:

  • Utilized Saccharomyces cerevisiae (yeast) as a model system.
  • Generated and analyzed yeast strains with a lys7Delta null mutation.

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  • Assessed SOD1 protein levels, copper incorporation, and enzymatic activity in vitro and in vivo.

    • Main Results:

    • Identified Saccharomyces cerevisiae LYS7 and human copper chaperone for SOD (CCS) as the soluble factors mediating copper delivery to SOD1.
    • Demonstrated that LYS7/CCS is specific for SOD1 and does not deliver copper to other cellular compartments.
    • Showed that yeast lacking LYS7 fail to incorporate copper into SOD1, rendering it inactive.
    • Confirmed that LYS7 and CCS restore holoSOD1 biosynthesis in vivo.

    Conclusions:

    • The LYS7/CCS pathway is essential for the copper-dependent activation of SOD1.
    • Understanding this pathway opens avenues for therapeutic strategies targeting SOD1-related diseases.
    • Targeting copper delivery to SOD1 may offer novel treatments for amyotrophic lateral sclerosis.