Comparison of Free Amino Acid Concentrations in Eosinophils and Neutrophils

Free amino acid concentrations were compared in eosinophils and neutrophils obtained from patients with eosinophilia. The level of glutamic acid was significantly higher in eosinophils than in neutrophils. This abundance of glutamic acid in eosinophils may be related to the formation of Charcot-Leyden crystals

Effect of Splenectomy on Free Amino Acid Concentrations in Erythrocytes of Patients with Hereditary Spherocytosis

Free amino acid concentrations in erythrocytes obtained from children with hereditary spherocytosis were measured before and after splenectomy. The concentrations of about half of the free amino acids were elevated before splenectomy, and this elevation may be due in part to increased numbers of reticulocytes. After splenectomy, however, the concentration of erythrocyte amino acids was not normalized and that of six to eight amino acids was still elevated. This elevation after splenectomy may be due to spherocytes themselves

Vitamin D and Cyclic Nucleotide Changes in Response to Calcitonin in Man

Vitamin D and cyclic AMP changes in response to calcitonin injection were studies in three normal adults and one patient with osteogenesis imperfecta. Plasma 1,25-(OH)2D levels increased in one normal adult and the patient. Plasma 25-OHD levels did not change. Plasma cyclic AMP was decreased in all four subjects, but urine cyclic AMP did not change. These results suggest that calcitonin may affect plasma cyclic AMP and may have some affect on renal la-hydroxylation in humans

Flow Cytometric Assessment of Neutrophil Oxidative Metabolism in Chronic Granulomatous Disease on Small Quantities of Whole Blood: Heterogeneity in Female Patients

A rapid and sensitive flow cytometric assay is presented for the quantitative estimation of the oxidative metabolic activity of individual polymorphonuclear leukocytes (PMN) on less than 100 td of whole blood. This procedure is a simplified version using whole blood of the method of Bass et al (J. Immunol. 130:1910, 1983) that estimated the metabolic burst activity of phorbol myristate acetate (PMA)-stimulated individual PMN as the intracellular generation of a fluorescence product by a flow cytometric assay. With this method, almost all the PMN from normal subjects responded to PMA as a single cell population generating bright intracellular fluorescence. PMN from a boy with chronic granulomatous disease (CGD), could not respond to PMA with any increase of their fluorescence intensity. His mother had two distinct PMN populations one functionally normal and the other defective, indicating a random lyonization in the carrier mother and the X-linked recessive mode of inheritance. In two female patients with CGD from unrelated families, their PMN responded to PMA, as a whole, with a minimal increase in the fluorescence intensity, but the metabolic defects in their PMN were not so complete as seen in a classical X-linked CGD boy. But, PMN from two female sibling patients from the other family responded to PMA as a single uniform cell population with a weak but definite fluorescence intensity. However, the genetic background of these female patients with CGD remains unclear, since PMN dysfunction could not be identified in their mothers with this method.This work was supported in part by a grant (No. 58440046) from the Ministry of Education of Japan

末梢血多核白血球の酸素消費能および付着能におよぼすノイロトロピンの影響

Effect of neurotropin (NSP) was studied on the oxygen consumption and adherence capacity of polymorphonuclear leukocytes of peripheral blood. Its therapeutic doses were found not to affect the phagocytic and adherent ability of leukocytes of healthy adults. In vitro experiments demonstrated that the respiration of leukocytes ceases much earlier in the presence of NSP than in its absence in a dose-dependent fashion, although the initial velocity of oxygen consumption associated with phagocytosis was unchanged. The fact that respiration tended to become normal when dialyzed neurotropin was used suggested that some inhibitory factors, which are dialyzable, are present in this preparation

慢性肉芽腫症患者好中球よりのリソゾーム酵素放出について

Lysosomal enzyme release from PMN exposed to STZ was examined using PMN from normal and CGD donors. Normal PMN showed an increase of extracellular lysosomal enzyme activity and a marked reduction of totol (extra-plus intra-cellular) enzyme activity after phagocytosis. On the other hand, in PMN from CGD patients, such a reduction of total enzyme activity was not observed and much more enzyme than normal was released extracellularly. When the supernatant from PMN from CGD patients after phagocytosis of STZ was incubated with an artificial H2O2 generating system, glucose plus glucose oxidase, the enzyme activities were greatly suppressed as the amount of H2O2 rose. A similar result was obtained with the addition of glucose oxidase during phagocytosis in PMN from CGD patients. These findings suggest that the presence of H2O2 might suppress lysosomal enzyme activities and result in inhibition of lysosomal enzyme release.This work was supported in parts by Grants-in-Aid for Scientific Research (Project Nos. 437010 and 544050) from the Ministry of Education, Science, and Culture of Japan

慢性肉芽腫症患児の多核白血球の膜電位変化

The activation of NADPH oxidase on the plasma membranes of polymorphonuclear leukocytes (PMNL) follows the change of membrane potential. It is thought that the activation of NADPH oxidase is disturbed in the PMNL of patients with chronic granulomatous disease (CGD). The change of membrane potential was examined in the PMNL of normal subjects and patients with CGD, with a lipophilic probe, di-O-C5(3). There was a disturbance in phorbol myristate acetate (PMA) and formyl-methionyl-leucyl-phenylalanine (FMLP)-induced depolarization but not in Ca ionophore A23187-induced depolarization of the PMNL of patients with CGD. A23187-induced depolarization was not always associated with O2- release in normal PMNL. A23187 differed from PMA and FMLP in several aspects of its action on depolarization. It is concluded that only depolarization which couples with O2- release is disturbed in the PMNL of patients with CGD. Increase in extracellular potassium (K) caused PMNL of both normal subjects and CGD patients to depolarize but made them not to release O2-. Otherwise, A23187-induced depolarization was disturbed in PMNL of CGD patients with higher extracellular K concentrations. These findings help to confirm the theory that K efflux on the plasma membrane is disturbed in the PMNL of patients with CGD.This work has been supported in part by Grants-in-Aid for Scientific Research (Project Nos. 544050, 448227) from the Ministry of Education, Science and Culture of Japan and a Grant-in-Aid from the Japan Medical Research Foundation

成人男性の血漿，赤血球，リンパ球，顆粒球における遊離アミノ酸の特徴的パターン

The concurrent concentrations of free amino acids in plasma, erythrocytes, lymphocytes,　and granulocytes are investigated in healthy men. The concentrations of most amino acids, except for aspartic acid which is much higher in erythrocytes, are more or less similar in plasma and erythrocytes. In lymphocytes the levels of taurine and aspartic acid are 100 and 200 times higher than the plasma levels, respectively, while in granulocytes the levels of taurine and aspartic acid are 300 and 100 times higher than the plasma levels, respectively. Aspartic acid in blood cells may play an important role common to the formed compartments of the blood, while taurine may play important roles in functions peculiar to leukocytes, especially in granulocytes.This work was supported in part by a Grant-in-Aid for Scientific Research (Project NO. 544050) from the Ministry of Education, Science, and Culture of Japan

血清中 1, 25-(OH)₂D 正常, 25-DHD 低値を示したくる病の一症例

In a patient with rickets developed after bowel surgery, we found a low serum concentration of 25-OHD and normal serum concentration of 1, 25-(OH)₂D. Oral administration of la-OHD₃ resulted in dramatic improvement of rickets and normalization of serum 25-OHD and calcium. These findings suggest that 25-OHD deficiency may play an important part in the pathogenesis of osteomalacia