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Science 7 January 1966:
Vol. 151. no. 3706, pp. 81 - 83
DOI: 10.1126/science.151.3706.81
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Articles

Chediak-Higashi Syndrome: Hereditary Gigantism of Cytoplasmic Organelles

Dorothy B. Windhorst 1, A. S. Zelickson 2, and Robert A. Good 3

1 Division-of Dermatology and Variety Club Heart Hospital, University of Minnesota, Minneapolis 55455
2 Division of Dermatology, University of Minnesota
3 Variety Club Heart Hospital, University of Minnesota

In the Chediak-Higashi syndrome, an anomalous hypopigmentation is associated with large lysosomal granules in the blood leukocytes. Since the inheritance pattern is that of an autosomal recessive trait, we postulated a common mechanism for these two primary features of the disease. Electron microscopy of melanocytes revealed that the pigmentary anomaly is indeed based on giant melanosomes. Since both types of granules, leukocytic and melanosomal, are characterized by limiting membranes, Chediak-Higashi disease may be a genetic disease of membranes.


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