Improvement of Chediak-Higashi leukocyte function by cyclic guanosine monophosphate.

Abstract

The addition of cholinergic agents and cyclic 3'5'-guanosine monophosphate (cGMP) to polymorphonuclear leukocytes in vitro from a patient with Chediak-Higashi syndrome corrected the impaired release of the lysosomal enzyme, beta-glucuronidase, to normal. Coinciding with the improvement in degranulation, the bactericidal capacity was enhanced to normal. Similar concentrations of cholinergic agents potentiated chemotaxis to control values. On the other hand, the phagocytic rate of lipopolysaccharide-coated paraffin-oil droplets was not altered by the cholinergic agents. The improvement in Chediak-Higashi syndrome polymorphonuclear leukocyte function by the addition of cholinergic agents and dibutyryl cGMP suggested disturbed intracellular cyclic nucleotide levels.

• Authors: L A Boxer, M Rister, J M Allen, R L Baehner
• Journal: Blood (Blood) Vol. 49 Issue 1 Pg. 9-17 (Jan 1977) ISSN: 0006-4971 [Print] United States
• PMID: 187266 (Publication Type: Case Reports, Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Glucuronidase
• Cyclic GMP

Topics

• Blood Bactericidal Activity
• Chediak-Higashi Syndrome (drug therapy)
• Chemotaxis, Leukocyte (drug effects)
• Cyclic GMP (therapeutic use)
• Female
• Glucuronidase (blood)
• Humans
• Infant
• Leukocytes (drug effects)
• Lysosomes (enzymology)