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  • 1
    Electronic Resource
    Electronic Resource
    Infrared studies of apatites. I. Vibrational assignments for calcium, strontium, and barium hydroxyapatites utilizing isotopic substitution (1974)
    s.l. : American Chemical Society
    Inorganic chemistry 13 (1974), S. 194-207 
    Details
    ISSN: 1520-510X
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1021/ic50131a039
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  • 2
    Electronic Resource
    Electronic Resource
    Infrared studies of apatites. II. Preparation of normal and isotopically substituted calcium, strontium, and barium hydroxyapatites and spectra-structure-composition correlations (1974)
    s.l. : American Chemical Society
    Inorganic chemistry 13 (1974), S. 207-214 
    Details
    ISSN: 1520-510X
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1021/ic50131a040
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Congenital absence of insulin cells in a neonate with diabetes mellitus and mutase-deficient methylmalonic acidaemia (1993)
    Springer
    Diabetologia 36 (1993), S. 352-357 
    Details
    ISSN: 1432-0428
    Keywords: Congenital diabetes mellitus ; absence of beta cells ; methylmalonic acidaemia ; mutase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report on a female neonate with diabetes mellitus and methylmalonic acidaemia, who died at age 16 days. Using immunocytochemistry, electron microscopy and in situ hybridisation, we were unable to demonstrate any insulin cells in the pancreatic islets. Methylmalonic acidaemia was caused by a methylmalonyl coenzyme A mutase apoenzyme defect. The metabolic crisis of the methylmalonic acidaemia aggravated the diabetes and may explain the failure of insulin therapy. Our results suggest that the infant suffered from a congenital absence of beta cells associated with a genetically transmitted mutase apoenzyme defect.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00400240
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  • 4
    Electronic Resource
    Electronic Resource
    Cadmium accumulation and toxicity in the kidney of the bay scallop Argopecten irradians (1981)
    Springer
    Marine biology 65 (1981), S. 35-43 
    Details
    ISSN: 1432-1793
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Abstract Specimes of bay scallops, Argopecten irradians, collected from shallow-water eel-grass beds near Beaufort, North Carolina (USA) in September 1979, were exposed to 0.7 ppm cadmium in flowing seawater for 5 d. This exposure resulted in a massive extrusion of the calcified concretions of most of the kidney epithelial cells, although marked morphological damage consisting of cytoplasmc degeneration was apparent in only a few focal areas of the kidney. In addition, unique cytoplasmic membrane-bound bodies were observed in epithelial cells of cadmium-treated but not control scallop kidneys. In some cells, these bodies appeared to fuse with the main concretion vacuole. Kidneys of cadmium-treated scallops accumulated cadmium to 200 ppm on a wet wt basis; of this 60% was associated with concretions (2 000 ppm dry wt) and 38% with the membranous pellet obtained after ultracentrifugation at 105 000 g for 1 h. Approximately 2% of total kidney cadmium was associated with the cytosolic fractions but, unlike zinc or manganese, which were bound to either high or low molecular weight species, a large component of the cadmium in this fraction was bound to a protein peak of approximately 21 000 daltons. Results of this study indicate that kidney concretions of A. irradians play a major role in the control of renal cadmium accumulation and excretion and hence the toxicity of cadmium to this organism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00397065
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  • 5
    Electronic Resource
    Electronic Resource
    Compositional, structural, and phase changes inin vitro laser-irradiated human tooth enamel (1984)
    Springer
    Calcified tissue international 36 (1984), S. 361-369 
    Details
    ISSN: 1432-0827
    Keywords: Laser-irradiated enamel ; Apatite modifications ; α-Tricalcium phosphate ; Tetracalcium phosphate ; Oxyhydroxyapatite
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine , Physics
    Notes: Summary Tooth enamel laser irradiated under certain conditions previously has been shown to have reduced subsurface demineralization rates. Identification of these laser-induced changes has bearing on understanding the dissolution rate reduction mechanism; some of these changes, ones that occur in high temperature regions, were studied in this report. X-ray diffraction and infrared spectroscopy were used to identify changes in enamel of extracted intact human teeth subjected to high energy density (∼10,000 J/cm2) 10.6 µm wavelength carbon dioxide laser irradiance. The laser irradiance melted the enamel apatite; this solidified melt was composed of minor phases of α-tricalcium phosphate, α-Ca3(PO4)2, and tetracalcium phosphate, Ca4(PO4)2O, and a major phase of modified apatite. The apatite modifications, as compared with the original were (1) reductions in contents of water, protein, carbonate, and chloride (or chloride rearrangement); (2) essentially no change in apatite hydroxide content; (3) possible incorporation of oxide replacing some hydroxide ions; and (4) an uptake of traces of carbon dioxide and cyanate. An infrared band at 434 cm−1 that appears in spectra of hydroxyapatite partially dehydroxylated by thermal treatment was assigned to oxide translation. This band was utilized to search for oxide formation in the laser-irradiated tooth enamel.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02405347
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  • 6
    Electronic Resource
    Electronic Resource
    Methionine synthase deficiency without megaloblastic anaemia (1997)
    Springer
    European journal of pediatrics 156 (1997), S. 925-930 
    Details
    ISSN: 1432-1076
    Keywords: Key words Methionine synthase ; 5 ; 10 Methylenetetrahydrofolate ; Megaloblastic anaemia ; Polymorphism ; Homocysteine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report findings on a child presenting with neonatal homocystinuria, hypomethioninaemia and severe neurological symptoms, including developmental delay and seizures. Methylmalonic aciduria was not present. The activity of methionine synthase in fibroblasts was severely deficient and formation of methylcobalamin from 57Co labelled cyanocobalamin was very low. The patients cells complemented with those of a cblE patient but not with those of two cblG patients. No biochemical or clinical response to injections of hydroxycobalamin was found. Both off treatment and on betaine and methionine supplementation the patient, at age 8 years, has not developed megaloblastic anaemia. In addition, the patient is homozygous for the C677T polymorphism in the 5,10 methylenetetrahydrofolate reductase (MTHFR) gene and the concomitant existence of this mutation with the methionine synthase defect may prevent folate «trapping» and thus anaemia. Conclusion We report the lack of megaloblastic anaemia in a patient with severe methionine synthase deficiency who is also homozygous for C677T in MTHFR, hypothesize that the MTHFR polymorphism protects the patient against anaemia and speculate that homozygosity for MTHFR C677T could cause the dissociation between haematological and neurological disease seen in some patients with vitamin B12 deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050744
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  • 7
    Electronic Resource
    Electronic Resource
    Late-onset holocarboxylase synthetase-deficiency: pre- and post-natal diagnosis and evaluation of effectiveness of antenatal biotin therapy (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 570-575 
    Details
    ISSN: 1432-1076
    Keywords: Key words Holocarboxylase synthetase deficiency ; Biotin therapy ; Prenatal diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The clinical and biochemical findings in a family with late-onset holocarboxylase synthetase (HCS) deficiency are described. The index patient had two life-threatening episodes of metabolic decompensation at the age of 13 and 18 months with ketotic hypoglycaemia, vomiting and progressive loss of consciousness. The child recovered without biotin therapy. Organic aciduria characteristic of multiple carboxylase deficiency (MCD) was found, however, the key metabolites were only slightly elevated in some samples. Biotinidase deficiency was considered but excluded by the finding of normal plasma biotinidase activity. The correct diagnosis was made only at the age of 19 months when severe MCD was found in lymphocytes in the presence of normal plasma biotin concentration. HCS deficiency was confirmed by fibroblast studies. Biotin therapy (20 or 40 mg/day) prevented further episodes and normalized biochemical parameters with so far normal development. During two subsequent pregnancies, 10 mg biotin/day was administered to the mother from the 20th week of gestation. At delivery plasma biotin in cord blood samples was 3–4 times higher than in maternal plasma. The 2nd child was unaffected. In the 3rd pregnancy prenatal diagnosis was performed at 16 weeks of gestation. The concentration of methylcitrate in amniotic fluid was within the normal range and that of 3-hydroxyisovalerate only slightly elevated. However, enzyme assays in cultured amniotic fluid cells were consistent with an affected fetus. At birth, carboxylase activities in lymphocytes of this newborn were only moderately decreased to 37% of mean normal. HCS deficiency was confirmed postnatally in fibroblasts. Development remains normal on biotin therapy (20 mg/day). Conclusion Prenatal diagnosis in families with milder forms of HCS deficiency has to be performed by enzyme assays in cultured amniotic cells since organic acid analysis of amniotic fluid may be inconclusive in affected fetuses. Biotin administered prenatally is effectively taken up by the fetus and prevents functional deficiency of the carboxylases in an affected newborn.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050881
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  • 8
    Electronic Resource
    Electronic Resource
    Biochemical diagnosis and outcome of 2 years treatment in a patient with combined methylmalonic aciduria and homocystinuria (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 818-820 
    Details
    ISSN: 1432-1076
    Keywords: Methylmalonic aciduria ; Homocystinuria ; Biochemical diagnosis ; Treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a patient with methylmalonic aciduria and homocystinuria due to a defect in cobalamin metabolism of the Cbl-C type mutant (McKusick 277400). Our case was diagnosed within the first 2 months of life by amino acid analysis (ion-exchange chromatography) and by biochemical studies in cultured fibroblasts ([14C]propionate incorporation, methionine and serine formation). We discuss the clinical course and the biochemical evolution after 2 years of hydroxycobalamin treatment that led to an improvement in general clinical condition and neurological performance.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957932
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  • 9
    Electronic Resource
    Electronic Resource
    Disorders of homocysteine metabolism (1998)
    Springer
    European journal of pediatrics 157 (1998), S. S39 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014301
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  • 10
    Electronic Resource
    Electronic Resource
    Genetic defects of folate and cobalamin metabolism (1998)
    Springer
    European journal of pediatrics 157 (1998), S. S60 
    Details
    ISSN: 1432-1076
    Keywords: Key words Folate ; Cobalamin ; Homocysteine ; Metabolism ; Disorders
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Deficient activity of an enzyme can result from a defect in the conversion of the vitamin to a co-enzyme as well from an abnormal apo-enzyme or disturbed binding of co-enzyme to enzyme. Conversion of dietary vitamin to intracellular active co-enzyme can be complex and require many physiological and biochemical processes including stomach release of bound vitamin, intestinal uptake, carriers/transport, blood transport, cellular uptake, intracellular release and intracellular compartmentalisation. Disorders of malabsorption (food cobalamin malabsorption, intrinsic factor deficiency and abnormal enterocyte cobalamin processing) and transport proteins (transcobalamin II deficiency, R-binder deficiency) mostly lead to disturbed function of the two cobalamin requiring enzymes, methylmalonyl CoA mutase and methionine synthase. Defects of early steps of intracellular cobalamin (cblF, cbl C/D) result in marked deficiencies of both cobalamin co-enzymes and homocystinuria combined with methylmalonic aciduria. Defective synthesis of adenosyl cobalamin in the cbl A/B defects leads to methylmalonyl CoA mutase. Isolated methionine synthase deficiency is also classified as a cobalamin disorder due to its associated deficient formation of methylcobalamin. Folate disorders include methylene-tetrahydrofolate reductase deficiency and glutamate formimino-transferase deficiency. In addition a hereditary disorder of intestinal folate transport has been described. Less well established are disorders of dihydrofolate reductase, methenyl-tetrahydrofolate cyclohydrolase, and defects of cellular folate uptake.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014306
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