Defective interleukin-1 production in a familial monocyte disorder with a combined abnormality of mobility and phagocytosis-killing.

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, CD14, CD33, Ia and LFA-1/Mac-1/p150,95 beta 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 immunodeficiency 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 less than 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 IL-1 production and the impaired phagocytic functions.     

Striking conservation of three extended HLA-DR13 haplotypes in the Japanese population

Abstract: HLA class II DNA typing was conducted for 1335 unrelated Japanese individuals. The study on the linkage disequilibrium revealed a striking conservation of HLA DR13 haplotypes. Among these Japanese, 155 were typed for HLA-DR13 serologically, and they were correspondent to three DRB1 alleles, DRB1*1301, 1302 and 1307 defined by using the polymerase-chain reaction and sequence-specific oligonucleotide probe (PCR-SSOP) method. The two alleles, DRB1*1301 and 1307 were exclusively associated with each specific DRB3-DQA1-DQB1 combination which was DRB1*1301-DRB3*0101-DQA1*0103-DQB1*0603, and DRB1*1307-DRB3*0202-DQA1*0501-DQB1*0301, respectively. DRB1* 1302, the most common DR13 allele in Japanese, had two significant associations with DRB3*0301-DQA1*0102-DQB1*0604 (DRB1*1302A) and with DRB3*0301-DQA1*0102-DQB1*0605 (DRB1*1302B). In this study, no other DR13 class II combinations were found. Ony the DRB1*1302A halotype was associated with the DPB1*0401 allele while the DRB1*1302B haplotype was not. The complete conservation of these DR13 class II haplotypes was found to extend toward the HLA class I region. They were HLA A3-B44-DRB1*1301, A33-B44-DRB1*1302A and A33-B17-DRB1*1302B. Japanese could be characterized with these three extended haplotypes which were remakrably different from those in Caucasian, Black and Asian other than Korean populations.     

Loss of J chain during primary immune responses in chicks.

J chain positive cells (JPC) were studied by direct immunofluorescence in embryonic and newly hatched chicks. The results indicated a decrease in the amounts of JPC in the embryonic spleen and bursa of Fabricius after in ovo antigenic stimulation with sheep erythrocytes (SRBC) compared with that of unstimulated lymphocytes. The level of thymic JPC in the control chicks and those subjected to antigenic stimulation was always about the same. Partial re-expression of the J chain in splenic lymphocytes was detected in newly hatched, antibody producing chicks, while the percentage of JPC in non-antibody producing chicks did not recover to the control level. Further evidence obtained indicated that the JPC decreases did not depend on the antigen dosage. After antigenic stimulation, J chain re-expression in cells of embryos and newly hatched non-antibody producing chicks was found to be essentially the same. These findings imply that the re-expression of J chain molecules is associated with immunoglobulin production. Furthermore, it seems plausible that the non-re-expression of the J chain occurred at the time of immunological unresponsiveness.     

J chain-positive cells in bursectomized chicks

Using embryonic chickens treated with testosterone propionate, the effects of congenital absence of the bursa of Fabricius determined by the frequency of J chain-positive cells was examined in the spleen, thymus and bone marrow at the embryonic and newly hatched stages. J chain-positive cells in the chicks without bursa were reduced in the spleen. No differences in the numbers of the cells were detected in the thymus and bone marrow. These results imply that removal of the bursa of Fabricius cannot entirely prevent the generation of J chain-positive B cells. Furthermore, these results partly suggest the important role of the bone marrow in the proliferation of some J chain-positive cells in chicks without bursa.     

Synchronous firing dynamics in a heterogeneous neurosecretory-cell population in an insect

Five pairs of neurosecretory cells in the subesophageal ganglion of the silkmoth Bombyx mori discharge action potentials in (near) synchrony to release a pheromone biosynthesis-activating neuropeptide (PBAN). Waveforms of compound action potentials recorded extracellularly from axonal tracts were analyzed to determine the firing activity, timing of spikes and the combination of active cells. Analyses revealed a heterogeneous cellular organization of the neurosecretory cell system. There was a gradient in the firing activity among the cells and the activity of a cell was closely related to relative timing of firing: the most active cell was usually the first to fire and participated in about 90% of all synchronous firing events, while the least active unit was mostly the last to fire and contributed to only 40% of all firing events. A cell with a higher firing activity had a higher potential to mediate propagation of synchronous firing in the cell system. Firing activities of right and left cell groups usually differed and the difference increased in case of a low temperature. Synchronous firings occurred more frequently among the same subgroup of cells rather than different subgroups. Heterogeneous cellular organization and coupling may be important for producing a graded pattern of active cell numbers, which seems to be suitable for maintaining a stable firing (secretory) activity of the cell system for a long period of time.     

The co-expression of ASIC3 with calcitonin gene-related peptide and parvalbumin in the rat trigeminal ganglion

The co-expression of ASIC3 with calcitonin gene-related peptide (CGRP) or parvalbumin (PV) was examined in the trigeminal ganglion (TG) by a double immunofluorescence method. ASIC3-immunoreactivity (IR) was detected in 23% of TG neurons. These neurons were of various sizes (range= 43-1768 microm(2), mean+/-S.D.=651+/-356 microm(2)); 26% and 14% of ASIC3-immunoreactive (IR) neurons co-expressed CGRP- and PV-immunoreactivity (IR), respectively; 33% and 13% of the TG neurons retrogradely labeled from the tooth pulp and facial skin, respectively, exhibited ASIC3-IR; 36% of CGRP-IR TG neurons which innervate these tissues co-expressed ASIC3-IR. Only 4% of ASIC3-IR cutaneous TG neurons showed PV-IR, while 25% of ASIC3-IR tooth pulp neurons were also immunoreactive for PV. The present study suggests that ASIC3-IR TG neurons supply the tooth pulp and facial skin with unmyelinated or finely myelinated axons. ASIC3-IR neurons which have large myelinated axons may be common in the tooth pulp but not the facial skin. The axonal morphology of ASIC3-IR TG neurons may depend on the variety of their receptive fields.