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November 1959

Some Properties of Brain Copper-Proteins in Hepatolenticular Degeneration

Author Affiliations

Department of Neurology, Neurosurgery and Psychiatry, New England Center Hospital; Department of Medicine (Neurology), Tufts University School of Medicine; Neurological Service, Massachusetts General Hospital, and Department of Neurology, Harvard Medical School.

AMA Arch Neurol. 1959;1(5):544-556. doi:10.1001/archneur.1959.03840050078011

This paper describes some properties of copper-protein combinations present in brains of patients with hepatolenticular degeneration and compares them with the properties of both naturally occurring and artificially created combinations of copper with proteins from normal human brain.

Hepatolenticular degeneration is characterized by a number of manifestations of abnormal copper metabolism.1 In particular, the marked increase in brain copper content2,3 and the improvement in neurological symptoms in some cases following administration of dimercaprol U. S. P. (BAL)4-10 suggest that the accumulation of copper in the brain may be the primary factor in the production of the central nervous system dysfunction. This concept of the pathogenesis of the disease, together with evidence that copper is also essential for normal nervous tissue function,11 has prompted us to investigate the types of organic combination in which copper is present in brains from cases of hepatolenticular degeneration and in normal

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