The Journal of Experimental Medicine, Vol 136, 644-649,
Copyright © 1972 by The Rockefeller University Press
THE HURLER SYNDROME: A STUDY OF CULTURED LYMPHOID CELL LINES
B. Shannon Danes 1,
T. H. Hütteroth 1,
H. Cleve 1, and
Alexander G. Bearn 1
1 From the Division of Human Genetics, Department of Medicine, Cornell University Medical College, New York 10021
Lymphoid suspension lines have been established from three patients with the Hurler syndrome and four normals. The Hurler lines can be distinguished from normals by (a) staining characteristics, (b) increase in total cellular mucopolysaccharide content, and (c) increase in dermatan sulfate. Hyaluronic acid is absent in cultured lymphoid cells from normal persons and patients with the Hurler syndrome. The availability of biochemically marked suspension cultures should prove useful for enzymatic studies as well as for further elucidation of this clinical syndrome.
Submitted on June 21, 1972