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  • 1
    Electronic Resource
    Electronic Resource
    Expression of the mutant gene for L-gulono-γ-lactone oxidase in scurvy-prone rats (1989)
    Springer
    Cellular and molecular life sciences 45 (1989), S. 126-129 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF01954844
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  • 2
    Electronic Resource
    Electronic Resource
    Decreased activity of cytochromec oxidase in the macular mottled mouse: an immuno-electron microscopic study (1989)
    Springer
    Acta neuropathologica 77 (1989), S. 465-471 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00687247
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  • 3
    Electronic Resource
    Electronic Resource
    Intracellular pH regulation in the mouse lacrimal gland acinar cells (1988)
    Springer
    The journal of membrane biology 101 (1988), S. 73-81 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF01872822
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  • 4
    Electronic Resource
    Electronic Resource
    Immunological and haematological changes during high-dose immunoglobulin therapy in an infant with autoimmune neutropenia (1987)
    Springer
    European journal of pediatrics 146 (1987), S. 412-415 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00444951
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  • 5
    Electronic Resource
    Electronic Resource
    Acetylcholine-induced change in intracellular Cl− activity of the mouse lacrimal acinar cells (1985)
    Springer
    Pflügers Archiv 405 (1985), S. 108-111 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00584530
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  • 6
    Electronic Resource
    Electronic Resource
    The potency of heparin-like activity of glycosaminoglycans released by human endothelial cells in culture (1985)
    Springer
    Cellular and molecular life sciences 41 (1985), S. 671-673 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF02007713
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  • 7
    Electronic Resource
    Electronic Resource
    Depletion of membrane phospholipid and mitochondrial dysfunction associated with coronary reperfusion (1985)
    Springer
    Basic research in cardiology 80 (1985), S. 241-250 
    Details
    ISSN: 1435-1803
    Keywords: phospholipase ; phospholipids ; mitochondria ; reperfusion injury ; canine heart
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The role of phospholipase (PLase) in the development of heart mitochondrial dysfunction following reperfusion was studied together with the effects of dilazep on the action of PLases and reperfusion injury. In vivo experiment: Seventy six adult mongrel dogs were divided into 3 groups; the control group (n=36), the dilazep 0.5 mg group (n=17) and the dilazep 1 mg group (n=23). Fifteen min after premedication with physiological saline or dilazep (0.5 mg/kg or 1 mg/kg), the left anterior descending coronary artery was occluded for 15 min and then reperfused for 5 min. Each group was further divided into two subgroups depending on the presence or absence of reperfusion arrhythmia. Immediately after 5 min of reperfusion, myocardial mitochondria were prepared from the normal and the reperfused areas. Pretreatment with dilazep induced a dose-dependent decrease in the incidence of reperfusion arrhythmia from 31% of the control to 24% (0.5 mg/kg) and 9% (1 mg/kg). In the arrhythmia cases in each group, functional deterioration of mitochondria from the reperfused area was observed with the increase in free fatty acids and the decrease in phospholipids in the reperfused mitochondria. In vitro experiment: Using L-α-dimyristoyl phosphatidylcholine as a substrate, myristic acid released by PLase A2 or by PLase C with or without pretreatment by dilazep was quantitatively determined. Dilazep inhibited the release of myristic acid by PLase A2 or by PLase C in a concentration-dependent manner. These results suggest that decomposition of mitochondrial membrane phospholipids caused by PLase activation following reperfusion was primarily responsible for the development of mitochondrial dysfunction, and that dilazep showed beneficial effects against reperfusion injury by inhibiting the action of PLases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01907900
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  • 8
    Electronic Resource
    Electronic Resource
    Applicability of Friedman plot (1986)
    Springer
    Journal of thermal analysis and calorimetry 31 (1986), S. 547-551 
    Details
    ISSN: 1572-8943
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Zusammenfassung Durch Erweiterung der ursprünglichen Gleichung wird gezeigt, daß die Friedman-Methode zur Bestimmung der Aktivierungsenergie chemischer Reaktionen unter Verwendung sowohl der Konversion als auch der Geschwindigkeit in thermoanalytischen Daten breite Anwendungsmöglichkeiten auf dem Gebiet des Wachstums von Kristallen aus Keimen, der Diffusion und anderer aus nur einem Grundprozeß bestehenden Vorgänge bietet.
    Abstract: Резюме Расширением первона чального уравнения, показано, что метод Фр идмэна для установления энерги и активации химическ их реакций с использованием пара метров превращения и скорос ти в термоаналитичес ких данных широко применимо к пр оцессу роста кристаллов на основе существующих центро в кристаллизации, к про цессу диффузии и друг им процессам, в которых и меет место единствен ный процесс.
    Notes: Abstract By expanding the initial equation, it is shown that the Friedman method for estimating the activation energy of chemical reactions by using both the conversion and the rate in the thermoanalytical data has wide applicability to crystal growth from pre-existing nuclei, diffusion and other processes in which a single unit process is involved.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01914230
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  • 9
    Electronic Resource
    Electronic Resource
    Clinical and molecular heterogeneity in patients with mitochondrial encephalomyopathies due to complex I deficiency (1988)
    Springer
    Journal of inherited metabolic disease 11 (1988), S. 333-336 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01800387
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  • 10
    Electronic Resource
    Electronic Resource
    Differently deleted mitochondrial genomes in maternally inherited chronic progressive external ophthalmoplegia (1989)
    Springer
    Journal of inherited metabolic disease 12 (1989), S. 359-362 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01799242
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