Deficiency of copper can cause neuronal degeneration.
The aim of this article is to emphasize the important role that copper plays in the function of nerve cells. We are reporting preliminary data which suggest that the swelling of axons which we produce in rats by iminodipropionitrile, IDPN, is due to its chelating action on copper, and how conversely supplementation with copper abolishes both symptoms and lesions. The copper values we obtained by atomic absorption spectrophotometry of the spinal cord and brain from the animals fully support this contention. In comparing these results with the diseases that are known to be due to copper deficiency, namely Menkes disease in man, swayback in lambs and several neurological mutant mice, we find not only similar axonal swellings, but also amelioration of symptoms and lesions by early administration of copper. Considering the main forms in which copper is present, we discuss the cuproproteins, i.e. ceruloplasmin and metallothionein, and their role in transport and delivery of copper to various organs. Further, the many cuproenzymes i.e. superoxide dismutase, tryptophan-2,3-dioxygenase, lysine oxidase, cytochrome oxidase, monoamine oxidases, tyrosinase, dopamine-beta-hydroxylase and d-amino levulinate dehydratase are noted for their roles in the nervous system. Finally, we suggest that neuronal copper deficiency should be more fully investigated as a possible etiological factor in the more common neurodegenerative diseases, such as Alzheimer's disease and amyotrophic lateral sclerosis, ALS.
Hartmann
H A
HA
Department of Pathology, University of Wisconsin, Madison 53705.
Evenson
M A
MA
D000818:Animals; D001921:Brain / Q000187:drug effects / Q000378:metabolism / Q000473:pathology*; D002614:Chelating Agents / Q000494:pharmacology*; D003300:Copper / Q000172:deficiency* / Q000378:metabolism / Q000494:pharmacology; D004195:Disease Models, Animal; D005260:Female; D008667:Metalloproteins / Q000378:metabolism; D009410:Nerve Degeneration / Q000187:drug effects*; D009570:Nitriles / Q000494:pharmacology*; D051381:Rats; D013116:Spinal Cord / Q000187:drug effects / Q000378:metabolism / Q000473:pathology*; D014131:Trace Elements / Q000378:metabolism
D002614:Chelating Agents; D008667:Metalloproteins; D009570:Nitriles; D014131:Trace Elements; C008110:3,3'-iminodipropionitrile; D003300:Copper