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  • 1
    Electronic Resource
    Electronic Resource
    s.l. : American Chemical Society
    Biochemistry 11 (1972), S. 1155-1160 
    ISSN: 1520-4995
    Source: ACS Legacy Archives
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-069X
    Keywords: Progressive systemic sclerosis ; Lysosomal hydrolases ; Fibroblast culture ; Collagen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In 14 patients with progressive systemic sclerosis (PSS) the activities of acid lysosomal glycosidases (α-, β-galactosidase, β-glucosidase, β-glucuronidase, and β-N-acetyl-glucosaminidase) were determined fluorometrically in serum, leukocytes, and skin tissue. The β-galactosidase was the only enzyme which exhibited a significantly elevated activity in PSS serum and skin but not leukocytes, as compared to the control. The activity patterns of the studied glycosidases in serum were similar to those found in skin, but differ from the distribution of glycosidase activities in leukocytes. In cultured dermal fibroblasts derived from PSS patients, an elevated intracellular activity of β-galactosidase was detected. These results suggest that the increased β-galactosidase activity in the serum originates from the skin fibroblasts.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Archives of microbiology 57 (1967), S. 93-110 
    ISSN: 1432-072X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Description / Table of Contents: Zusammenfassung 1. Mit Chrysosporium keratinophilum, Chr. evolceanui, Arthroderma quadrifidum, A. curreyi und Anixiopsis stercoraria haben wir Untersuchungen über Waschstum, Morphologie des Haarbefalls, Keratinolyse und Enzymausscheidung bei Kultur der Pilze in einer mineralischen Basisnährlösung mit Haarpartikeln als Kohlenstoff-Stickstoff-Quelle während einer Versuchsdauer von 77 Tagen ausgeführt. 2. Alle diese Pilze griffen Haar mit sogenannten Perforationsorganen, senkrecht zur Haarachse vordringenden, keiflörmigen Mycelzapfen, und nicht mit sogenannten Bohrhyphen, ebenfalls senkrecht eindringenden, schmalen, geraden Hyphen an. Daneben kamen auch oberflächliche Läsionen der Haarrinde und Lockerung ihrer Struktur vor. Die Haarbefallsform durch diese apathogenen, keratinophilen Bodenpilze entspricht also der vieler Dermatophyten. 3. Das Ausmaß des Keratinabbaues durch die apathogenen Bodenpilze war zwar unterschiedlich (26–49% des gegebenen Keratins) dabei aber keinesfalls niedriger als durch pathogene Dermatophyten (28–40%). 4. Die Pilze veränderten den pH-Wert der Nährlösung (Ausgangswert 5,2) zum Schwachalkalischen hin (Endwert 8,1). pH 7 wurde bereits 14–21 Tage nach Versuchsbeginn erreicht. Diese Alkalisierungstendenz ist ein typisches Merkmal proteino- besonders keratinophiler Pilze. 5. Alle Pilze schieden Proteinasen aus, deren Aktivität/mg Mycel keine direkte Korrelation zur Intensität der Keratinolyse erkennen ließ. In der Diskussion der Ergebnisse haben wir dieses Phänomen und seine vermutlichen Ursachen ausführlich besprochen. 6. In den Kulturfiltraten sämtlicher Pilze haben wir alkalische Phosphatase (pHopt. 8,5) nachgewiesen. Die Aktivität/mg Mycel war für die einzelnen Pilzarten resp.-stämme relativ spezifisch. Da in keratinisiertem Milieu freies Phosphat im allgemeinen nur in Spuren vorhanden ist, vermitteln Phosphatasebestimmungen Hinweise auf potentielle Existenzchancen der Pilze in Keratinen.
    Notes: Summary 1. Chrysosporium keratinophilum, Chr. evolceanui, Arthroderma quadrifidum, A. curreyi and Anixiopsis stercoraria were cultivated for 77 days in a medium containing hair particles as the sole source of carbon and nitrogen and mineral salts. During this time growth, hair penetration, keratinolysis and excretion of enzymes by these fungi were observed. 2. All these fungi attacked the hair by means of so called penetration organs, wedge-shaped columns of short wide cells, but not by so called borers or boring hyphae, narrow hyphae without any septa. Besides penetration organs we found cortical erosions and destruction of the hair cortex. Hair penetration therefore took place in the same manner by apathogenic keratinophilic soil fungi and by dermatophytes. 3. Hair digestion by apathogenic soil fungi and pathogenic dermatophytes was nearly on the same level (26–49% of the given keratin). 4. These fungi were able to alter the pH of the medium from 5.2 at the begin to 8.1 at the end of the experiments. pH 7 was reached 14–21 days after inoculation. The alcalinizing tendency characterizes proteino- and especially keratinophilic fungi. 5. There is no direct correlation between the activity of the proteolytic enzymes (activity per mg dry weight; substrate gelatine) and the break-down of keratin by these fungi. The probable causes of this phenomenon are discussed. 6. Alcaline phosphatase was found in the culture broth. The phosphatatic activity per mg dry weight of the mycelium was on a distinct level for each of these species.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 149 (1990), S. 574-576 
    ISSN: 1432-1076
    Keywords: Fructose-1,6-diphosphatase deficiency ; Gluconeogenesis ; Hypoglycaemia ; Neonatal hyperbilirubinaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three boys and one girl suffering from inherited fructose-1,6-diphosphatase (FDPase) deficiency are reported. All four patients had less than 25% residual hepatic FDPase activity. While in two out of three patients the enzyme deficiency was also expressed in leucocytes, one patient had a normal enzyme activity. Remarkably, three patients had pronounced neonatal hyperbilirubinaemia requiring exchange transfusion.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Key words: Hereditary metabolic disorders – Selective screening – Collaborative study
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Since the early 1970s selective screening for inherited metabolic disorders has been performed in larger childrens hospitals or metabolic centres of the former East-Germany. As a rule the following methods were employed: initially paper chromatography, drop, dip and spot tests, later on thin-layer chromatography and more recently enzyme analysis, gas chromatography, mass spectrometry and HPLC. Normally urine, blood or leucocytes were investigated. The diagnoses were confirmed in metabolic centres in Greifswald, Berlin or Leipzig or in collaboration with specialized laboratories abroad. About 130 000 subjects from former East Germany as well as from different East European countries were investigated, of which 365 patients were diagnosed and classified into roughly 40 various metabolic diseases. The proportion of positive diagnoses was 1 in 400.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: Hereditary metabolic disorders ; Selective screening ; Collaborative study
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Since the early 1970s selective screening for inherited metabolic disorders has been performed in larger childrens hospitals or metabolic centres of the former East-Germany. As a rule the following methods were employed: initially paper chromatography, drop, dip and spot tests, later on thin-layer chromatography and more recently enzyme analysis, gas chromatography, mass spectrometry and HPLC. Normally urine, blood or leucocytes were investigated. The diagnoses were confirmed in metabolic centres in Greifswald, Berlin or Leipzig or in collaboration with specialized laboratories abroad. About 130000 subjects from former East Germany as well as from different East European countries were investigated, of which 365 patients were diagnosed and classified into roughly 40 various metabolic diseases. The proportion of positive diagnoses was 1 in 400.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Physics Letters A 24 (1967), S. 59-60 
    ISSN: 0375-9601
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Physics
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 106 (1982), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The activities of five lysosomal hydrolases were determined fluorometrically in the serum of patients with systemic sclerosis (PSS), systemic lupus erythematosus (SLE), dermatomyositis (DM), rheumatoid arthritis (RA), or Raynaud's disease (RD). In PSS the β-galactosidase activity was significantly increased compared with controls and the other connective tissue diseases. The β-N-acetyl-glucosaminidase was significantly increased in PSS, SLE and DM. In PSS both enzymes were more active in the early stage of the disease than later. These changes of enzyme pattern seem to be a relatively reliable marker for the differential diagnosis of PSS compared to other connective tissue diseases, especially for RD, in which the β-galactosidase activity was significantly decreased. Further work is required to determine whether these polysaccharide-degrading acid hydrolases play a role in the pathogenesis of PSS.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 99 (1978), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: No tree anti-basement membrane antibodies were found in the sera of four patients with bullous pemphigoid, and no free anti-DNA antibodies in the sera of two patients with systemic lupus erythematosus. The sera were treated with 3 mol/l urea and by gel chromatography to separate these antibodies from any circulating complexes, and antibodies to basement membrane were detected by immunofluorescence and to DNA by the Farr technique. The appropriate antibodies were found in all the sera, indicating that failure to detect antibodies by routine procedures may be due to binding of the antibodies to soluble antigens, forming complexes in the sera.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 0014-5793
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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