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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 42 (1964), S. 613-618 
    ISSN: 1432-1440
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Biochemical details following the intoxication with mushrooms (amanita phalloides) are reported in two italian cousines with heterocygotic glutathion reductase deficiency in red cells. 1. The enzymatic activities of the TPNH dependant glutathion reductase were 5,6, respectively 6.1 IU/1011 erythrocytes (normal values 13.2±1.6 IU). 2. The instability of GSH and the formation of Heinz bodies in the red cells were markedly pronounced, even in the absence of a decompensated metabolic acidosis. 3. Together with the recovery from the intoxication the GSH instability and the Heinz body formation by acetyl phenyl hydrazine became normal, as is seen in all cases with heterocygotic glutathion reductase deficiency. 4. In both cases the red cells had a normal carbohydrate metabolism and a normal content of methemoglobin. However the intracellular potassium values were lowered. During the intoxication with mushrooms the ATP content of the red cells was unchanged.
    Notes: Zusammenfassung Es wird über biochemische Veränderungen bei zwei italienischen Cousinen mit heterozygotem Glutathionreduktasemangel in Erythrocyten unter der Intoxikation mit Pilzen (wahrscheinlich Amanita phalloides) berichtet. 1. Die TPNH-abh. Glutathionreduktaseaktivitäten betrugen 5,6 bzw. 6,1 IE/1011 Erythrocyten (Normalwerte 13,2±1,6 IE). 2. Die GSH-Instabilität und Heinzkörperbildung in den Erythrocyten waren trotz des Fehlens einer dekompensierten metabolischen Acidose offenbar wegen des Glutathionreduktasemangels sehr ausgeprägt. 3. Mit dem Abklingen der Intoxikationserscheinungen normalisierten sich auch die GSH-Instabilität und die Heinzkörperbildung durch Acetylphenylhydrazin, wie es bei allen heterozygoten Glutathionreduktasemangelträgern ohne Intoxikation beobachtet wird. 4. Der Kohlenhydratstoffwechsel und Methämoglobingehalt waren bei beiden Kranken normal, während der Kaliumgehalt der roten Blutzellen herabgesetzt war. Auch der ATP-Gehalt fiel unter der Pilzintoxikation in den Erythrocyten nicht ab. 5. Unter dem Einfluß der Pilztoxine kam es bei einer Patientin als Ausdruck der Leberschädigung zu einer vorübergehenden Erhöhung der Transaminase-und Lactatdehydrogenaseaktivitäten im Serum mit Umkehr des De Ritis-Quotienten. 6. Im Aminosäurechromatogramm des Serums, das bei derselben Patientin angefertigt wurde, war die Argininkonzentration deutlich herabgesetzt.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 49 (1971), S. 1151-1152 
    ISSN: 1432-1440
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 56 (1978), S. 291-295 
    ISSN: 1432-1440
    Keywords: Leber ; Harnstoffzyklus-Enzyme ; Steroide ; Liver ; Urea-cycle enzymes ; Steroids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Activities of Krebs-Henseleit enzymes were determined in liver biopsies of normal persons and in patients suffering from alcoholic hepatitis and chronic active hepatitis. Prednisone was administered for five days in falling dosis (1.5 mg–0.5 mg/kg/body weight) to patients with alcoholic hepatitis and to controls. Patients with chronic active hepatitis received 15–20 mg prednisone daily for more than three months. In healthy persons prednisone did not influence the activities of Krebs-Henseleit enzymes. In patients with alcoholic hepatitis most of the urea-cycle enzymes are significantly decreased (p〈0.05) when compared to controls. After glucocorticoid administration enzyme activities remained unchanged. Activities of most of the urea-cycle enzymes are significantly (p〈0.05) decreased in untreated patients with chronic active hepatitis. In some of these patients, glucocorticoid administration was associated with a remission as proved by clinical, biochemical and histological data. Activities of the rate-limiting enzymes of the ureacycle (ASAS, CPS) increased significantly in these patients. By contrast, alterations of enzyme activities could not be observed in patients who failed to respond favourably to steroid treatment.
    Notes: Zusammenfassung Die Aktivitäten der Enzyme des Harnstoffzyklus wurden in Leberbiopsien von Normalpersonen, von Patienten mit alkoholischer Fettleber-Hepatitis und chronisch-aggressiver Hepatitis bestimmt. Prednison wurde 5 Tage vor der Biopsie in fallender Dosierung (1,5 mg-0,5 mg/kg/Körpergewicht) verabreicht an Patienten mit alkoholischer Fettleber-Hepatitis und an Lebergesunde. Patienten mit chronisch-aggressiver Hepatitis standen unter einer Steroidtherapie (15–20 mg Prednison/tgl.) für mehrere Monate. Die Aktivitäten der Harnstoffzyklus-Enzyme wurden bei lebergesunden Personen durch Steroide in der genannten Dosis nicht verändert. Bei Patienten mit alkoholischer Hepatitis waren die meisten Harnstoffzyklus-Enzyme signifikant erniedrigt (P〈0,05) im Vergleich zu lebergesunden Kontrollpersonen. Die Gabe von Glucocorticoiden veränderte die Enzymaktivitäten nicht. Die Aktivitäten der meisten Harnstoffzyklus-Enzyme sind signifikant (p〈0,05) erniedrigt bei (unbehandelten) Patienten mit chronisch-aggressiver Hepatitis. Bei einigen dieser Patienten konnte nach Glucocorticoidgabe eine Remission der Erkrankung aufgrund klinischer, biochemischer und histologischer Parameter nachgewiesen werden. Bei diesen Patienten kam es zu einem signifikanten Anstieg der geschwindigkeitslimitierenden Enzyme des Harnstoffzyklus (ASAS und CPS). Dagegen blieb der Anstieg der Enzymaktivitäten aus bei solchen Patienten, welche trotz Steroidgaben nicht in eine — auch histologisch nachweisbare — Remission kamen.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1440
    Keywords: Urea cycle ; Enzymes ; Intracellular localisation ; Liver ; Harnstoffcyclusenzyme ; Intrazelluläre Lokalisation ; Leber
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Enzyme des Krebs-Henseleit-Harnstoffcyclus wurden durch Differentialzentrifugation und fraktionierte Extraktion in Rattenleber und in menschlicher Leber lokalisiert. Argininosuccinatlyase (ASAL) und Argininosuccinatsynthetase (ASAS) sind Enzyme der löslichen Cytoplasmafraktion. Ornithin-Ketosäure-Transaminase (OKT), Carbamyl-Phosphat-Synthetase (CPS) und Ornithin-Carbamyl-Transferase (OKT) sind in der Kern- und Mitochondrienfraktion der Leberzelle lokalisiert. Der größte Teil der Arginase-Aktivität ist an subcelluläre Strukturen (möglicherweise an Kerne) gebunden. Ein kleiner Teil der Arginase-Aktivität wurde in der löslichen Cytoplasmafraktion gefunden. Die Enzyme des Krebs-Henseleit-Harnstoffcyclus sind in der Rattenleber und in der menschlichen Leber identisch verteilt. Unterschiedliche Resultate hinsichtlich der subzellulären Lokalisation mitochondrialer Enzyme des Krebs-Henseleit-Cyclus in der menschlichen Leber beruhen auf der Schädigung von Mitochondrienmembranen während der Gewebspräparation und nicht auf unterschiedlichen in-vivo Bedingungen.
    Notes: Summary Enzymes of the Krebs-Henseleit urea-cycle were localized by means of differential centrifugation and fractional tissue extraction in rat liver and in human liver. Argininosuccinatlyase (ASAL) and Argininosuccinatsynthetase (ASAS) represent enzymes of the soluble cytoplasmic fraction. Ornithine-ketoacid-transaminase(OKT), carbamyl-phosphate-synthetase (CPS) and ornithine-carbamyl-transferase (OCT) are localized in the mitochondrial and nuclei fractions of the liver cell. Most of the arginase activity is bound to subcellular structures (probably to nuclei). A small portion of arginase-activity was found in the soluble cytoplasmatic fraction. The enzymes of the Krebs-Henseleit urea-cycle are equally distributed in rat liver and in human liver. Differences in the subcellular localisation of (mitochondrial) enzymes in human liver could be attributed to mitochondrial breakage during tissue preparation and do not represent in-vivo conditions.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 57 (1979), S. 151-159 
    ISSN: 1432-1440
    Keywords: Glycoproteins ; Glycosyltransferases ; Lectins ; Oncology ; Immunology ; Glycoproteine ; Glycosyltransferassen ; Lectine ; Onkologie ; Immunologie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Zunehmende chemische und biochemische Kenntnisse über Struktur, Biosynthese und Katabolismus der Glycopteine haben auch neue Einblicke in die Pathobiochemie und klinische Bedeutung der Glycoproteine ermöglicht. Diese werden in der vorliegenden Übersicht zusammenfassend dargestellt. 1. Während der Fetalperiode besitzen die endständigen Kohlenhydratreste der Glycoproteine wichtige Funktionen bei der Zell-Adhäsion, -Fusion und gerichteten Migration. Daneben wird die Rolle der endständigen N-Acetylneuraminsäure (NANA) von Glycoproteinen für die immunologische Toleranz zwischen Mutter und Kind diskutiert. 2. Unter onkologischen Gesichtspunkten wird der Einfluß des Glycoproteinmusters der Plasmamembran für das Tumorwachstum und die Metastasierung beschrieben. Veränderte Glycosyltransferase-Aktivitäten im Serum von Tumorpatienten gewinnen zunehmend an diagnostischer Bedeutung. 3. Durch endständige Kohlenhydratreste von Glycoproteinen wird nicht nur die Immunogenität von Makromolekülen, sondern auch die Immunkompetenz des Organismus beeinflußt. Hierdurch erlangen diese Strukturen sowohl bei der immunologischen Tumorabwehr als auch bei Autoimmunerkrankungen klinische Relevanz. Über Ergebnisse ausgewählter Fachgebiete wird in einer nachfolgenden Übersicht berichtet (Klin. Wochenschr.57, 199 (1979)
    Notes: Summary Increasing knowledge on structure, biosynthesis and catabolism of glycoproteins have given new insights on the patho-biochemical and clinical significance of these macromolecules. The most important results and conclusions are summarized in this review. 1. The terminal sugars of glycoproteins—N-acetylneuraminic acid (NANA) and L-fucose—as well as the penultimate galactose molecule have important functions in cell interaction, adhesion and recognition. Moreover, these carbohydrates mediate the migration and distribution of cells and it is believed that they are essential part of the feto-maternal “immunological barrier”. 2. Evidence indicating that the composition and pattern of plasma membrane glycoproteins is associated with tumour growth and metastatic formation is accumulating. Moreover, the determination of serum glycosyltransferase activity is gaining increasing interest, because the level of theses enzymes is substantially elevated in patients with neoplastic disease. 3. Diseases of the autoimmunosystem are likely linked to a disturbed glycoprotein metabolism. The clinical importance is underlined by studies on immunotherapy of tumours.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 61 (1983), S. 277-283 
    ISSN: 1432-1440
    Keywords: Tryptophan loading test ; Liver function
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Because of its specific hepatic degradation tryptophan was orally administered (50 mg/kg) to patients with various chronic liver diseases (n=30) and to healthy volunteers (n=8) as a test for hepatic function. The plasma half life of tryptophan was determined between 4 and 8 h after the amino acid load. It was found that in patients with cirrhosis (n=25) the half life of tryptophan was prolonged to 4.7±0.4 h (x±SD), compared to 2.0±0.1 h in the controls. The tryptophan half life also correlated with the plasma concentration of albumin, bilirubin, cholinesterase and prothrombin time in these patients. In addition a significant correlation was observed with the galactose elimination capacity and the 45 min retention of BSP. Thus, the oral tryptophan loading test may be suitable for a more specific determination of functional impairment of the liver in chronic liver disease. In decompensated cirrhotic patients alterations of the tryptophan metabolism seen to be related to indicators of hepatic encephalopathy. The test may therefore be used to assess the degree and risk of hepatic encephalopathy in such patients.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 64 (1986), S. 590-594 
    ISSN: 1432-1440
    Keywords: Tryptophan-metabolism ; Tryptophan ; Liver cirrhosis ; Hepatic encephalopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Tryptophan is considered to be one of the agents involved in the pathogenesis of hepatic encephalopathy. In our study, we evaluated tryptophan metabolism in liver disease. A bolus of 1.5 g ofl-tryptophan was administered intravenously to 14 patients with noncirrhotic liver disease, 40 patients with liver cirrhosis, and 8 healthy volunteers. As pharmacokinetic parameters, the half life, clearance, and volume of distribution of free and total tryptophan were determined using a biexponential formula. In addition, the activity of liver tryptophan pyrrolase, the key enzyme of tryptophan metabolism, was measured in liver biopsy specimens of 15 patients with noncirrhotic liver disease, 8 patients with cirrhosis of the liver, and 4 patients with histologically normal livers. Healthy subjects and patients with noncirrhotic liver disease both showed similar results in measured and calculated data. In contrast, patients with cirrhosis revealed significant alterations of the pharmacokinetic parameters of free and total tryptophan: the half-life was increased by 195% and 176%, the clearance was decreased by 73% and 34%, respectively, and the activity of tryptophan pyrrolase was decreased by 22%. The tryptophan transfer in cirrhosis amounted to only 0.75±0.03 g per 24 h compared with 2.6±0.34 g per 24 h in healthy individuals. The findings demonstrate that patients with cirrhosis show a marked reduction in their ability to metabolize tryptophan. This should be taken into account in the oral and parenteral nutrition of those patients.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1440
    Keywords: Ascites ; Liver cirrhosis ; Plasminogen ; Antiproteases ; Fibrinolysis ; Dexamethasone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Fibrinolysis induced by the infusion of plasminogen activators into the circulation has been shown to cause coagulation disorders in ascites retransfusion. Dexamethasone is known to inhibit the synthesis of plasminogen activators by peritoneal macrophages. We therefore assessed its potential in preventing the occurrence of fibrinolysis by injecting 16 mg dexamethasone intraperitoneally in 10 patients 24 h before ascites retransfusion was performed. In addition, the effect of dexamethasone upon the activity or concentration of several proteases and antiproteases related to coagulation in plasma and ascites was analyzed on 15 occasions. An increase of the activity of plasminogen, α2-antiplasmin, and antithrombin III, and in the concentration of α1-protease inhibitor in ascites was induced by the dexamethasone injection. However, the reaction was not identical in all patients. Those patients having an increase of plasminogen activities of 0.6 CTA U/ml or more did not show signs of fibrinolysis during retransfusion. The results obtained indicate that intraperitoneal injection of dexamethasone decreases the concentration of plasminogen activators in ascites and thereby reduces the risk of coagulation disorders during retransfusion procedures. Since the effect is variable and not sustained, assessment of preoperative plasminogen concentrations is mandatory in order to prevent complications.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 50 (1972), S. 887-889 
    ISSN: 1432-1440
    Keywords: Chronic liver diseases ; hyperammonemia ; urea cycle ; argininosuccinate synthetase (ASAS) ; Chronische Lebererkrankungen ; Hyperammonämie ; Harnstoffcyclus ; Argininosuccinatsynthetase (ASAS)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In der vorliegenden Arbeit wird eine Methode zur direkten Bestimmung der Aktivität der Argininosuccinatsynthetase (ASAS) im Homogenat eines Leberpunktates angegeben. Die Enzymaktivität wird bei 10 Gesunden und bei 13 Patienten mit chronischen Lebererkrankungen (Lebercirrhose 9, chronisch-aggressive Hepatitis 4) im Lebergewebe bestimmt. Bei den chronischen Lebererkrankungen ist die Aktivität der ASAS gegenüber Gesunden hoch-signifikant erniedrigt. Die ASAS ist das limitierende Enzym im Harnstoffcyclus; die starke Aktivitätsminderung des Enzyms in der Leber läßt auf eine Störung dieses Reaktionscyclus schließen und erklärt die bei chronischen Lebererkrankungen, insbesondere Lebercirrhosen, nachgewiesene Konzentrationszunahme von Citrullin und Ammoniak im Blutplasma.
    Notes: Summary A method for the direct determination of argininosuccinate synthetase (ASAS) activity in liver homogenates is described. The activity of the enzyme was determined in bioptic material from 13 patients suffering of different chronic liver diseases and from 10 healthy controls. The results show a highly significant decrease of ASAS activity in the livers of the patients as compared to the healthy controls. This decrease indicates that the urea cycle is impaired in chronic liver disease. The observation explains the increase in serum citrulline and ammonia concentrations in chronic hepatic disease.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 56 (1978), S. 835-837 
    ISSN: 1432-1440
    Keywords: Human liver: Biochemistry ; Synthesis of purine nucleotides ; Benzbromarone ; Menschliche Leber: Biochemie ; Purinnucleotid-Synthese ; Benzbromaronum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wurden die Aktivitäten der PP-Ribose-P-Amidotransferase und der Hypoxanthin- und Adenin-Phosphoribosyltransferasen im 100 000 × g-Überstand von menschlichen Leberzellen bestimmt. Sowohl die wesentlich höheren spezifischen Aktivitäten der Enzyme des „salvage-pathways“ als auch die wesentlich niedrigeren Km-Werte für PP-Ribose-P weisen darauf hin, daß unter normalen Bedingungen der Reutilisierungsstoffwechsel der bevorzugte Purinnucleotidsyntheseweg ist. In Übereinstimmung mit unseren bisherigen Ergebnissen wurde kein Einfluß von Benzbromaronum auf die untersuchten Enzyme des Purinnucleotidstoffwechsels festgestellt.
    Notes: Summary 5-Phosphoribosyl-1-pyrophosphate amidotransferase and the hypoxanthine-guanine phosphoribosyltransferases (H-PRT, A-PRT) were assayed in human liver cells and some of the properties of these enzymes were determined. The Michaelis-Menten constants of both H-PRT and A-PRT for phosphoribosylpyrophosphate are at least an order of magnitude lower than for human PP-ribose-P amidotransferase. A the enzyme level the higher specific activities of phosphoribosyltransferases would, in effect, give precedence to purine salvage in human liver. In addition, no effect of benzbromaronum on enzyme activities could be demonstrated.
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