Granulocyte function in the Chediak-Higashi syndrome of mice.

Granulocyte function was evaluated using activity of S. aureus and group D strepperitoneal exudate granulocytes from a tococcus by CHS cells through 90 mm of instrain of beige mice with the Chediak-Hicubation. The bactericidal defect was most gashi syndrome (CHS). Defective granupronounced at early time periods and was locyte chemotaxi of CHS cells (43% related to impaired intracellular killing. normal) was documented. There was no abThese defects of CHS mice granulocytes normality in the ability of CHS serum to were analogous to those reported in man function as a chemotactic stimulus. and it is concluded that the CHS mouse is a Phagocytosis of ‘4C-labeled Staphylococcus most convenient and representative model aureus by CHS granulocytes was normal, of the CHS of man. However, there was reduced bactericidal T HE CHEDIAK-HIGASHI SYNDROME (CHS) in man is a rare autosomal recessive disease characterized by partial oculocutaneous albinism, frequent pyogenic infections, neutropenia, and characteristic “giant” lysosomal granules present in all granule-containing cells.’ The disease has also been described in Aleutian mink,2 a strain of partial albino Hereford cattle,3 and recently in the beige mouse.4’5 Susceptibility to infection in man is associated with granulocytopenia,#{176} an abnormality of granulocyte chemotaxis,7 a delay in the killing of phagocytized bacteria, particularly staphylococci, and an abnormal distribution of enzymes in leukocyte lysosomes.9 When beige mice were recently found to have increased susceptibility to infection caused by Staphylococcus aureus, Streptococcus pneumoniae, and Candida albicans,’#{176} studies to further characterize their leukocyte function were undertaken with the hope of establishing a convenient laboratory model of CHS.