The copper chaperone CCS is abundant in neurons and astrocytes in human and rodent brain

Jeffrey D. Rothstein, Margaret Dykes-Hoberg, Laura B. Corson, Mark Becker, Don W. Cleveland, Donald L. Price, Valeria Cizewski Culotta, Phillip C. Wong

Research output: Contribution to journalArticlepeer-review

100 Scopus citations


Copper trafficking in mammalian cells is highly regulated. CCS is a copper chaperone that donates copper to the antioxidant enzyme copper/zinc superoxide dismutase 1 (SOD 1). Mutations of SOD1 are responsible for ~20% of familial amyotrophic lateral sclerosis (FALS). Monospecific antibodies were generated to evaluate the localization and cellular distribution of this copper chaperone in human and mouse brain as well as other organs. CCS is found to be ubiquitously expressed by multiple tissues and is present in particularly high concentrations in kidney and liver. In brain and spinal cord, CCS was found throughout the neuropil, with expression largely confined to neurons and some astrocytes. Like SOD1, CCS immunoreactivity was intense in Purkinje cells, deep cerebellar neurons, and pyramidal cortical neurons, whereas in spinal cord, CCS was highly expressed in motor neurons. In cortical neurons, CCS was present in the soma and proximal dendrites, as well as some axons. Although the distribution of CCS paralleled that of SOD1, there was a 12-30-fold molar excess of SOD1 over CCS. That both SOD1 and CCS are present, together, in cells that degenerate in ALS also emphasizes the potential role of CCS in mutant SOD1-mediated toxicity.

Original languageEnglish (US)
Pages (from-to)422-429
Number of pages8
JournalJournal of Neurochemistry
Issue number1
StatePublished - 1999


  • Amyotrophic lateral sclerosis
  • CCS
  • Copper chaperone
  • Motor neuron
  • Superoxide dismutase

ASJC Scopus subject areas

  • Biochemistry
  • Cellular and Molecular Neuroscience


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