Granulocyte Function in the Chediak-Higashi Syndrome of Mice

Granulocyte function was evaluated usingperitoneal exudate granulocytes from astrain of beige mice with the Chediak-Higashi syndrome (CHS). Defective granulocyte chemotaxis of CHS cells (43%normal) was documented. There was no abnormality in the ability of CHS serum tofunction as a chemotactic stimulus.Phagocytosis of ¹⁴C-labeled Staphylococcusaureus by CHS granulocytes was normal.However, there was reduced bactericidalactivity of S. aureus and group D streptococcus by CHS cells through 90 min of incubation. The bactericidal defect was mostpronounced at early time periods and wasrelated to impaired intracellular killing.These defects of CHS mice granulocyteswere analogous to those reported in manand it is concluded that the CHS mouse is amost convenient and representative modelof the CHS of man.

Submitted on April 24, 1973 Revised on July 26, 1973 Accepted on August 1, 1973