Biomedical Sciences ETDs

Publication Date

7-3-1979

Abstract

The McLeod erythrocyte is characterized by a weak Kell antigenicity, lack of Kx antigen, and an abnormal cell morphology. These properties are shared by the prepositus, Hugh McLeod, and male children afflicted by type II chronic granulomatous disease. X-linked chronic granulomatous disease involves the inability of affected leukocytes to digest phagocytized bacteria leading to premature death. Prior studies have shown the McLeod erythrocyte membrane to be not only antigenically abnormal, but also exhibit an abnormal acanthocytic shape and decreased water permeability. These properties were found to be independent of the membrane phospholipid composition, cholesterol/phospholipid ratio, and microviscosity. The decreased water permeability, abnormal morphology, and weak Kell antigenicity seen in McLeod seem to suggest the involvement of an abnormal membrane protein or glycoprotein. This prompted the present study aimed at investigating membrane protein composition by two investigative techniques, double replication freeze fracture electron microscopy and discontinuous polyacrylamide gel electrophoresis. Discontinuous polyacrylamide gel electrophoresis showed no apparent band additions or deletions in comparing the electrophoretic profiles of cell membrane protein from McLeod and normal red blood cells. The McLeod erythrocyte did show a statistically significant increase in the number of intramembraneous particles revealed by double replication freeze fracture as compared to normals. These particles are thought to be partially composed of protein and present evidence suggest they may be composed jointly of the band 3 polypeptide and glycophorin complex. Evidence exists which implicates either or both of these polypeptides in membrane water transport. Hence, in the McLeod phenotype, a structural abnormality of the erythrocyte membrane parallels its functional defect.

Document Type

Thesis

Language

English

Degree Name

Biomedical Sciences

Level of Degree

Masters

Department Name

Biomedical Sciences Graduate Program

First Committee Member (Chair)

William Raleigh Galey, Jr.

Second Committee Member

Albert Ratner

Third Committee Member

Andrew Paul Evan

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