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  • 1
    Electronic Resource
    Electronic Resource
    Origin of the enhanced activity of lysosomal β-galactosidase in serum and skin in progressive systemic sclerosis (1987)
    Springer
    Archives of dermatological research 279 (1987), S. 299-302 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00431221
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  • 2
    Electronic Resource
    Electronic Resource
    Biochemical and clinical observations in four patients with fructose-1,6-diphosphatase deficiency (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 574-576 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF01957696
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  • 3
    Electronic Resource
    Electronic Resource
    Results of selective screening for inborn errors of metabolism in the former East Germany (1994)
    Springer
    European journal of pediatrics 153 (1994), S. S14 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF02138771
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  • 4
    Electronic Resource
    Electronic Resource
    Results of selective screening for inborn errors of metabolism in the former East Germany (1994)
    Springer
    European journal of pediatrics 153 (1994), S. S14 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF02138771
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  • 5
    Electronic Resource
    Electronic Resource
    Acid lysosomal hydrolases in systemic sclerosis and other connective tissue diseases (1982)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 106 (1982), S. 0 
    Details
    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
    URL: http://dx.doi.org/10.1111/j.1365-2133.1982.tb04554.x
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  • 6
    Electronic Resource
    Electronic Resource
    Detection of antibodies after immune complex splitting in serum of patients with bullous pemphigoid and systemic lupus erythematosus (1978)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 99 (1978), S. 0 
    Details
    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
    URL: http://dx.doi.org/10.1111/j.1365-2133.1978.tb07057.x
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  • 7
    Electronic Resource
    Electronic Resource
    Reaction mechanisms and control properties of phosphotransferases - A report of a Joint Biochemical Symposium of the All-Union Biochemical Society of the USSR and the Biochemical Society of the GDR, Schloss Reinhardsbrunn, GDR, May 3-7, 1971
    Amsterdam : Elsevier
    FEBS Letters 17 (1971), S. 1-5 
    Details
    ISSN: 0014-5793
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0014-5793(71)80548-3
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  • 8
    Electronic Resource
    Electronic Resource
    Pathogenesis of Progressive Systemic Sclerosis (1986)
    Oxford, UK : Blackwell Publishing Ltd
    International journal of dermatology 25 (1986), S. 0 
    Details
    ISSN: 1365-4632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-4362.1986.tb02244.x
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  • 9
    Electronic Resource
    Electronic Resource
    Biochemistry of liver development in the perinatal period (1983)
    Springer
    Cellular and molecular life sciences 39 (1983), S. 473-483 
    Details
    ISSN: 1420-9071
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Just before birth, changes occur in the metabolic capacities of rat liver so that the animal can adapt to changes in the substrate supply. In utero, glucose is the main energy-generating fuel and the liver metabolism is directed towards glucose degradation. The activities of the rate-limiting enzymes of glycolysis, hexokinase and phosphofructokinase, are high. In preparation for post-natal life, when the continuous glucose supply from the mother is interrupted, very large amounts of glycogen are stored in the late fetal liver. With the intake of the fat-rich and carbohydrate-poor milk diet, the animal develops the ability to synthesize glucose de novo from non-carbohydrate precursors. During suckling, metabolic energy is derived mainly from the β-oxidation of fatty acids, which in turn is an essential prerequisite for the high rate of gluconeogenesis, by yielding acetyl-CoA for the activation of pyruvate carboxylase and by generating a high NADH/NAD ratio for the shift of the glyceraldehyde 3-phosphate dehydrogenase reaction in the direction of glucose formation.-The developmental adaptation of metabolism and the process of enzymatic differentiation are closely connected with the maturation of the endocrine system and the changes in the concentration of circulating hormones. The neonatal regulation of phosphoenolpyruvate carboxykinase and of tyrosine aminotransferase by variations in the hormonal milieu around birth, and also the interacton of hormonal and nutritional factors in the induction of serine dehydratase and glucokinase at the end of the suckling period, will be discussed in detail.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01965164
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  • 10
    Electronic Resource
    Electronic Resource
    Automatische Wirkstoff bestimmungen in Arzneimittelzubereitungen (1972)
    Springer
    Fresenius' Zeitschrift für analytische Chemie 259 (1972), S. 363-366 
    Details
    ISSN: 1618-2650
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Zusammenfassung Es wird eine automatische Lipasebestimmung in Festal® mit dem AutoAnalyzer® angegeben. Eine in einem Emulsionsrührer in Gegenwart von Aktivatoren und einem Emulgator hergestellte Triolein/ Wasseremulsion wird mit einem Dragee-Extrakt der zu bestimmenden Probe inkubiert. Das bei der enzymatischen Spaltung unter anderem entstehende Glycerin wird mit Perjodat zu Formaldehyd oxydiert, der sich mit zugegebenem Ammoniak und Acetylaceton in einer Hantzsch-Kondensation zum 3,5-Diacetyl-1,4-dihydrolutidinumsetzt. Das Reaktionsprodukt wird fluorimetrisch ausgemessen. Die relative Standardabweichung der Methode beträgt ±3,5%, die Analysenfrequenz 20 Einzelbestimmungen/h.
    Notes: Abstract An automatic determination of lipase in Festal® by aid of the AutoAnalyzer® is described. A triolein/water emulsion made by aid of a special emulsifier, is incubated with a sample of the drug to be determined. Glycerol, one of the products gained by the enzymic reaction, is oxidized by periodate into formaldehyde; the addition of ammonia and acetylacetone to the formaldehyde produces in a Hantzsch condensation 3,5-diacetyl-1,4-dihydrolutidine, which is measured fluorimetrically. The relative deviation of this method is ±3.5%, the frequency of analysis is 20 determinations/h.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00423898
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