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  • 1
    Electronic Resource
    Electronic Resource
    Oviposition-deterring pheromone inRhagoletis cerasi L.: Purification and determination of the chemical constitution (1987)
    Springer
    Cellular and molecular life sciences 43 (1987), S. 157-164 
    Details
    ISSN: 1420-9071
    Keywords: Tephritidae ; Rhagoletis cerasi L ; oviposition-deterring pheromone ; constitution ; purification ; electrophysiology ; contactchemoreceptors ; oviposition behavior ; fast atom bombardment mass spectrometry ; two-dimensional mass spectrometry ; gas chromatography ; mass spectrometry ; 1H-NMR spectroscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary An oviposition-deterring pheromone (ODP) of the European cherry fruit flyRhagoletis cerasi L. was isolated from faeces using cellulose and several reverse phase TLC and HPLC procedures. The biological activity was evaluated by means of behavior tests and by electrophysiological recordings from tarsal contact chemoreceptors. The compound was structurally characterized as a N[15(β-glucopyranosyl)oxy-8-hydroxypalmitoyl]-taurine by spectroscopic means. The configurations of C-8 and C-15 of the fatty acid constituent remain to be established by synthetic work.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01942834
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Defective neutrophil and monocyte functions in glycogen storage disease type Ib: A literature review (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 33-38 
    Details
    ISSN: 1432-1076
    Keywords: Glycogen storage disease type Ib ; Polymorphonuclear leukocytes ; Neutrophils ; Monocytes ; Phagocytic dysfunction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A summary review of leukocyte function in 42 published cases of glycogen storage disease Ib is presented. Polymorphonuclear and monocyte dysfunctions were evidenced in the majority of cases, whereas lymphocytes appeared to be unaffected. Phagocyte dysfunctions comprised in vivo mobilization and motility, in vitro random and directed migration, and one or several component functions of the “metabolic” (“respiratory”) burst. On the basis of the available data it is impossible to know whether a primary functional deficit of the glucose 6-phosphate transport protein of the microsomal glucose 6-phosphatase system, as demonstrated in liver, also exists in these phagocytic cells and is responsible for this dysfunction.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02072085
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Liver glycogen synthase deficiency: a rarely diagnosed entity (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 561-567 
    Details
    ISSN: 1432-1076
    Keywords: Key words Glycogen deficiency ; Glycogen synthase ; Ketotic ; hypoglycaemia ; Corn (maize) ; starch ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three children from two German families are described and the observations compared with the previously published three families comprising eight patients. The two index cases presented with morning fatigue, had ketotic hypoglycaemia when fasting which rapidly disappeared after eating, and hepatic glycogen deficiency and absent or very low hepatic glycogen synthase activity. Metabolic profiles comprising glucose, lactate, alanine, and ketones in blood were typical for hepatic glycogen synthase deficiency. Symptoms were rapidly relieved and chemical signs corrected by introducing frequent protein-rich meals and night-time feedings of suspensions of uncooked corn (maize) starch. The discovery of oligosymptomatic and asymptomatic siblings suggests that there are more persons with undiagnosed hepatic glycogen synthase deficiency. Conclusion Liver glycogen synthase deficiency is likely to be more common than is believed today. It should be sought in children who, before the first meal of the day, present with drowsiness, lack of attention, pallor, uncoordinated eye movements, disorientation or convulsions and who have hypoglycaemia and acetone in urine.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957905
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Partial deficiency of galactose-1-phosphate uridyltransferase (1995)
    Springer
    European journal of pediatrics 154 (1995), S. S40 
    Details
    ISSN: 1432-1076
    Keywords: Galactosemia ; Duarte variant ; Compound heterozygosity ; Newborn screening
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In screening programmes testing newborns for galactose-1-phosphate uridyltransferase and/or galactose, partial enzyme deficiency is frequently discovered. This is shown for one laboratory in Switzerland where 104 newborns were singled out from a total of 476000. Of these, 72 had partial transferase deficiency below 9 μmol/h per g Hb and were assumed to be compound heterozygotes for “classical” galatosemia and the Duarte variant. Present day management of compound heterozygotes consisting of lactose-free diet for 4 months is described and discussed in the light of published opinion. The appropiateness of this pragmatic approach can at present not be judged according to objective criteria.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02143802
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Clinical, biochemical and molecular findings in a patient with X-linked liver glycogenosis followed for 40 years (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 919-923 
    Details
    ISSN: 1432-1076
    Keywords: Key words Glycogen storage disease type IX ; Liver ; Phosphorylase kinase ; X-linked
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Phosphorylase kinase (PHK) is a regulatory enzyme in glycogen metabolism. Mutations in the gene encoding the α subunit of PHK (PHKA2) have been shown to be responsible for X-linked liver glycogenosis (XLG). XLG, a frequent type of glycogen storage disease, is characterised by hepatomegaly and growth retardation. Two subtypes of XLG have been described: XLG type I patients have a clear-cut PHK deficiency in liver and blood cells, whereas XLG type II patients have a normal or residual activity. Here, we present clinical, biochemical and molecular findings on a liver glycogenosis patient in whom the diagnosis XLG II only became clear after enzyme assays in the liver and identification of the disease-causing mutation. A missense mutation replacing arginine at amino acid position 186 by histidine (R186H) was identified in the PHKA2 gene. Mutations of the same arginine residue have been previously found in at least four other unrelated XLG II patients. Conclusion Arginine at position 186 of the α subunit seems to play an important role in the structure or the regulation of PHK. In patients with XLG having normal or residual PHK activity where XLG II is suspected, the identification of mutations in PHKA2 leads to the final classification.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050967
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    Paper (German National Licenses)
    Fulltext
  • 6
    Electronic Resource
    Electronic Resource
    Liver glycogen synthase deficiency: A rarely diagnosed entity (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 561-567 
    Details
    ISSN: 1432-1076
    Keywords: Glycogen deficiency ; Glycogen synthase ; Ketotic hypoglycaemia ; Corn (maize) starch ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three children from two German families are described and the observations compared with the previously published three families comprising eight patients. The two index cases presented with morning fatigue, had ketotic hypoglycaemia when fasting which rapidly disappeared after eating, and hepatic glycogen deficiency and absent or very low hepatic glycogen synthase activity. Metabolic profiles comprising glucose, lactate, alanine, and ketones in blood were typical for hepatic glycogen synthase deficiency. Symptoms were rapidly relieved and chemical signs corrected by introducing frequent protein-rich meals and night-time feedings of suspensions of uncooked corn (maize) starch. The discovery of oligosymptomatic and asymptomatic siblings suggests that there are more persons with undiagnosed hepatic glycogen synthase deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957905
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
  • 7
    Electronic Resource
    Electronic Resource
    Lysosomal enzyme activities in serum and leukocytes from patients with carbohydrate-deficient glycoprotein syndrome type IA (phosphomannomutase deficiency) (1998)
    Springer
    Journal of inherited metabolic disease 21 (1998), S. 167-172 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract From 10 patients with carbohydrate-deficient glycoprotein (CDG) syndrome due to phosphomannomutase (PMM) deficiency, out of 10 lysosomal enzymes, 7 enzyme activities were measured in serum and 9 in leukocytes. In serum there was a 2-fold to 4-fold increase in activity of β-glucuronidase, β-hexosaminidase, β-galactosidase, and arylsulphatase A. In leukocytes, however, several enzymes had reduced activity, particularly α-fucosidase, β-glucuronidase and α-mannosidase. These abnormalities could result from missorting, defective reuptake and/or reduced stability of the enzymes due to the defective glycosylation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1005351927573
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    Paper (German National Licenses)
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