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  • 1
    ISSN: 1432-0533
    Keywords: Menkes kinky hair syndrome ; Macular mottled mouse ; Mitochondrion ; Cytochromec oxidase ; Gold-labeling immuno-electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The macular mottled mouse is a murine model of the kinky hair syndrome, characterized by a deficiency in copper transport. Cytochromec oxidase (CCO), a respiratory enzyme, is located in the inner mitochondrial membrane and consists of seven subunits, along with copper and iron. Biochemical and histochemical findings indicated that CCO activity was decreased in the cerebellum of the macular mottled mice but not in that of the controls. Immunocytochemical analysis, using anti-CCO and anti-complex III rabbit sera, revealed that CCO in the macular mottled mice was stained more weakly than that in the controls. Immuno-electron microscopic examination of CCO and complex III, using a method of gold labeling, was also performed. In the control mice, a high concentration of gold particles present over CCO and complex III could be seen in the inner mitochondrial membrane. The number of CCO-labeled gold particles was remarkably less, however, in the macular mottled mice, while no significant difference was found in the labeling of complex III between the two groups. It may concluded that the very low CCO enzyme content in the macular mottled mouse results not only from a copper transport disorder but also from a CCO protein synthesis disorder which impairs the localization of CCO protein in the cerebellum.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Cellular and molecular life sciences 45 (1989), S. 126-129 
    ISSN: 1420-9071
    Keywords: L-gulono-γ-lactone oxidase ; ascorbic acid deficiency ; enzyme defect ; rat ; nuclei acid hybridization
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary A mutant strain of Wistar rats with L-gulono-γ-lactone oxidase deficiency has recently been established. To investigate this deficiency by DNA and RNA blot hybridization analyses, a fragment of a previously cloned cDNA encoding rat L-gulono-γ-lactone oxidase was used as a probe. When genomic DNA of the mutant rat was digested with several restriction enzymes, the probe hybridized to fragments of the same sizes as those produced from DNA of normal rats. Poly(A)+RNA from the liver of the mutant rat was found to contain an L-gulono-γ-lactone oxidase-specific mRNA of a normal size at a comparable level to that of normal rats. An in vitro translation experiment revealed that the mRNA programmed the synthesis of an enzyme protein which had the same molecular weight as that of the translational product of the normal mRNA, although the amount synthesized was markedly reduced as compared with that synthesized with the normal mRNA. In accordance with this observation, a very low but definite degree of L-gulono-γ-lactone oxidase activity was detected in the microsomes of the mutant rat by a newly developed, highly sensitive method.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Cellular and molecular life sciences 41 (1985), S. 671-673 
    ISSN: 1420-9071
    Keywords: Endothelial cells ; glycosaminoglycan ; heparin, smooth muscle cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Glycosaminoglycans isolated from culture medium conditioned by human endothelial cells showed heparin-like antithrombin III cofactor activity measured by Xa inhibition. Their activity was relatively weak, 0.1% of the potency of heparin, but was approximately 5-fold more potent than that of glycosaminoglycans derived from vascular smooth muscle cells.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Autoimmune neutropenia ; High-dose immunoglobulin therapy ; Antineutrophil autoantibodies ; Flow cytometry neutrophil-bound IgG
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract High-dose immunoglobulin (HD-Ig) therapy was given to an infant with autoimmune neutropenia (AIN), and antineutrophil autoantibodies (ANAA). The patient's absolute neutrophil count in peripheral blood increased from 300/ mm3 to 3000–4000/mm3 7 days after treatment. Although the neutrophil count gradually decreased thereafter, transient increases were observed after each single booster infusion repeated at 3-week intervals. By continuing this treatment, clinical symptoms were markedly alleviated, and the patient's susceptibility to infection was reduced. The increase in neutrophils showed a positive correlation with the increase in serum IgG, and with the increase in the ratio of T helper/T suppressor cells. The neutrophil-bound IgG level and serum lysozyme level were decreased after HD-Ig therapy.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-2013
    Keywords: Lacrimal gland ; Cl− activity ; Acetylcholine ; Cl− permeability ; Ca2+ ionophore
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Using double-barreled Cl−-sensitive microelectrodes, intracellular Cl− activity (A Cl i ) in the mouse lacrimal acinar cells in vitro was determined in both resting and secretory phases. In the resting stateA Cl i was 31 mmol/l which was 1.4 times higher than that predicted for the passive distribution according to the membrane potential (V m) of −41 mV. Addition of acetylcholine (ACh, 1μM) hyperpolarizedV m to −63 mV and decreasedA Cl i to 20 mmol/l which was still twice the equilibrium activity. A-23178 produced similar changes inV m andA Cl i to those induced by ACh. It was concluded that Cl− was actively accumulated in the acinar cells and, in the secretory phase, Cl− efflux was enhanced by the increased driving force and Ca2+-mediated increase in the Cl− permeability across the cell membrane.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    The journal of membrane biology 101 (1988), S. 73-81 
    ISSN: 1432-1424
    Keywords: intracellualr pH ; lacrimal gland ; amiloride ; DIDS ; Na+/H+ antiport ; Cl−/HCO 3 − ; antiport
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology
    Notes: Summary Intracellular pH (pH i ) of the acinar cells of the isolated, superfused mouse lacrimal gland has been measured using pH-sensitive microelectrodes. Under nonstimulated condition pH i was 7.25, which was about 0.5 unit higher than the equilibrium pH. Alterations of the external pH by ±0.4 unit shifted pH i only by ±0.08 unit. The intracellular buffering value determined by applications of 25mm NH 4 + and bicarbonate buffer solution gassed with 5% CO2/95% O2 was 26 and 46mm/pH, respectively Stimulation with 1 μm acetylcholine (ACh) caused a transient, small decrease and then a sustained increase in pH i . In the presence of amiloride (0.1mm) or the absence of Na+, application of ACh caused a significant decrease in pH i and removal of amiloride or replacement with Na+-containing saline, respectively, rapidly increased the pH i . Pretreatment with DIDS (0.2mm) did not change the pH i of the nonstimulated conditions; however, it significantly enhanced the increase in pH i induced by ACh. The present results showed that (i) there is an active acid extrusion mechanism that is stimulated by ACh; (ii) stimulation with ACh enhances the rate of acid production in the acinar cells; and (iii) the acid extrusion mechanism is inhibited by amiloride addition to and Na+ removal from the bath solution. We suggest that both Na+/H+ and HCO 3 − /Cl− exchange transport mechanisms are taking roles in the intracellular pH regulation in the lacrimal gland acinar cells.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 11 (1988), S. 333-336 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Documenta ophthalmologica 26 (1969), S. 192-201 
    ISSN: 1573-2622
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé Sur le muscle droit supérieur du lapin anesthésié on a enregistré simultanément des potentiels EMG et des potentiels intracellulaires lents prenant naissance dans des foyers cellulaires multiples. Quelques enregistrements ont montré l'apparition synchrone de potentiels EMG et de potentiels intracellulaires lents ‘multi-foyers’; dans tous ces cas les potentiels EMG étaient essentiellement monophasiques et d'une durée supérieure à 4 msec, présentant ainsi des caractéristiques différentes de celles d'un potentiel de pointe propagé. Il a pu être vérifié que les fibres musculaires qui donnent naissance aux potentiels intracellulaires lents multifoyers, sont des fibres à innervation multiple. L'étude des potentiels lents chez l'homme pourrait être intéressante.
    Abstract: Zusammenfassung Im Rectus superior des Kaninchens wurden in Narkose gleichzeitig EMG Potentiale und spontane langsame, intracelluläre multifokale Potentiale registriert. Die EMG Potentiale, die mit den intracellulären Potentialen synchron auftreten, sind hauptsächlich monophasisch, sie dauern wenigstens 4 msec und unterscheiden sich deutlich von den ‘Spike’-Potentialen. Es hat sich bestätigt, daß die Fasern, welche solche spontanen, intracellulären, multifokalen langsamen Potentiale hervorrufen, tatsächlich eine mehrfache Innervation besitzen. Es wäre interessant, auch beim Menschen diese langsamen Potentiale zu untersuchen.
    Notes: Summary EMG potentials were recorded simultaneously with spontaneous intracellular multifocus slow potentials in the rabbit superior rectus under general anesthesia. Those EMG potentials which were synchronous with such intracellular potentials, are essentially monophasic and at least 4 msec in duration, clearly different from spike potentials. It was verified, that the fibers which produce such spontaneous intracellular multifocus slow potentials are indeed multiply innervated. The study of such slow potential activity in human extraocular muscles may be profitable.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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