We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Defective interleukin-1 production in a familial monocyte disorder with a combined abnormality of mobility and phagocytosis-killing.
- Authors
Komiyama, A.; Ichikawa, M.; Kanda, H.; Aoyama, K.; Yasui, K.; Yamazaki, M.; Kawai, H.; Miyagawa, Y.; Akabane, T.
- Abstract
Monocytes in a familial monocyte disorder, a recently recognized primary immunodeficiency syndrome, with impaired phagocytic functions were studied for their ability to produce interleukin 1 (IL-1) as well as the surface property. Monocytes from two children (siblings) with the disorder possessed CD11b, CD13, CDI4, CD33. Ia and LFA-l/Mac-l/p150,95β subunit antigens as determined by flow cytometry. Electron microscopic cytochemistry showed that the monocytes had surface glycoproteins reactive with four representative lectins. The IL-1 production by monocytes was assayed in the two patients and compared with that in six children with primary immunodeficieney syndromes and some monocyte abnormalities; three had congenital neutropenia, two had hyper-IgE syndrome, and one had defective monocyte chemotaxis. Monocyte culture supernatants were prepared with stimulation by lipopolysaccharide or silica, and their IL-1 activity was measured by the mouse thymocyte-proliferation assay. The patients' monocytes were defective in IL-1 production: the values were < 1.0% of the control monocyte values (n = 12) and were in contrast with those of congenital neutropenia monocytes of 186.2% to 204.3%. These results demonstrate a familial monocyte disorder which is characteristic among the immunodeficiency syndromes with regard to the defective lL-1 production and the impaired phagocytic functions.
- Subjects
IMMUNOLOGICAL deficiency syndromes; NEUTROPENIA; MONOCYTES; PHAGOCYTOSIS; IMMUNE response; CYTOMETRY
- Publication
Clinical & Experimental Immunology, 1988, Vol 73, Issue 3, p500
- ISSN
0009-9104
- Publication type
Article