Several pedigrees of which some members showed a clinical syndrome consisting of mental changes, choreatic involuntary movements, limb muscles atrophy, and acanthocytosis have been reported in the United States and the United Kingdom. Such a case and some of the family members who had such abnormalities as acanthocytosis, hypo-beta-lipoproteinemia, convulsions, and confusion was observed. Results of biochemical analysis of catecholamines and their metabolites in CSF and urine showed an elevated value of norepinephrine in CSF and increased urinary secretion of DOPAC. The authors propose to designate this syndrome an amyotrophic chorea with acanthocytosis.

Athetosis↗

PubMed1980

The effects of copper and copper. o-phenanthroline complex on the intact human erythrocytes.

In the presence of albumin, cytotoxic effects of copper on erythrocytes in vitro were reduced. The presence of o-phenanthroline, on the other hand, accelerated deleterious effects of copper on the erythrocytes resulting in a decrease in GSH level, accumulation of copper in the cell, loss of potassium and gain of sodium, cross-linking of membrane proteins mainly involving spectrin and band 3, and echinocyte formation. These changes were not reversible. Prior treatment of the cells with N-ethylmaleimide inhibited the cross-linking of membrane proteins and echinocytic transformation by copper o-phenanthroline complex (CuP or diamide to a certain extent. Treatment of the cells with vinblastine, a stomatocytogenic agent, restored biconcave disc shape of CuP-treated erythrocytes, although it did not inhibit the cross-linking of spectrin. Blocking of sulfhydryl groups was not the prerequisite for the copper to be accumulated in the erythrocyte. These results may be helpful for the understanding of the mechanism of hemolysis associated with copper intoxication in vivo.

Copper↗

PubMed1980

Shape changes of GSH-treated erythrocytes during complement-induced hemolysis.

Shape changes of GSH-treated human erythrocytes during the acidified serum test were examined by a differential interference microscope. A time-course study of the erythrocytes undergoing complement-mediated hemolysis revealed two kinds of hemolytic processes. One is rapid hemolysis, in which GSH-treated erythrocytes are transformed into spherical forms prior to hemolysis. The other process is gradual or slow hemolysis of a small proportion of GSH-treated erythrocytes. In this process, the erythrocytes are transformed into echinocytes and then into spheroechinocytes prior to hemolysis. However, echinocytic shape changes are not an absolute prerequisite to hemolysis, since echinocytes are not always transformed into a prelytic spherical form, but some of them are reversed to discocytes. Under the experimental conditions of this study, rapid hemolysis of spherical erythrocytes is the main mechanism of hemolysis.

Complement System Proteins↗

PubMed1980