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  • 11
    Electronic Resource
    Electronic Resource
    Transsulphuration and methylation of homocysteine in control and mutant human fibroblasts
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Molecular Cell Research 721 (1982), S. 201-207 
    Details
    ISSN: 0167-4889
    Keywords: (Human fibroblast) ; Amino acid metabolism ; Homocysteine ; Methylation ; Transsulfuration
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0167-4889(82)90069-6
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  • 12
    Electronic Resource
    Electronic Resource
    Inhibition of human and murine cytosolic epoxide hydrolase by group-selective reagents
    Amsterdam : Elsevier
    Comparative Biochemistry and Physiology -- Part B: Biochemistry and 104 (1993), S. 299-308 
    Details
    ISSN: 0305-0491
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0305-0491(93)90372-C
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  • 13
    Electronic Resource
    Electronic Resource
    The crystal structure of calcium succinate monohydrate (1994)
    Springer
    Journal of chemical crystallography 24 (1994), S. 437-440 
    Details
    ISSN: 1572-8854
    Keywords: Ca(C4H4O4)·H2O ; pentagonal pyramid ; calcium succinate ; crystal structure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geosciences , Physics
    Notes: Abstract The crystal structure of calcium succinate monohydrate, Ca(C4H4O4)·H2O, has been determined by single crystal X-ray diffraction. The crystals are monoclinic witha=11.952(2),b=9.691(2),c=11.606(2)Å, β=108.81(1)°, space group C2/c,Z=8,V=1272.49 Å3,d m =1.80, andd c =1.818 Mg m−3. The structure was refined by full-matrix least-squares techniques toR=0.027,R w =0.040, for 829 reflections with1≥3δ(I). Ca is coordinated to seven oxygen atoms, and the coordination polyhedron is best described as a pentagonal bipyramid. One carboxylate group in the succinate ion is bonded to three different Ca ions, forming a four-membered chelate ring with one Ca ion is bonded to three different Ca ions, forming a four-membered chelate ring with one Ca ion and unidentate bridge bonds to two other Ca ions. The other carboxylate group is bonded to two Ca ions through unidentate bonds. The structure is highly polymeric. The general structural features are nearly identical to those of calcium adipate monohydrate.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01666091
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  • 14
    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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  • 15
    Electronic Resource
    Electronic Resource
    Mechanisms of low temperature epitaxial silicon growth using ArF excimer laser photochemical vapor deposition from disilane
    Amsterdam : Elsevier
    Thin Solid Films 218 (1992), S. 48-57 
    Details
    ISSN: 0040-6090
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Physics
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0040-6090(92)90902-N
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  • 16
    Electronic Resource
    Electronic Resource
    Organochlorine pesticides and polychlorinated biphenyls in the Arctic Ocean food web (1992)
    Springer
    Archives of environmental contamination and toxicology 22 (1992), S. 41-54 
    Details
    ISSN: 1432-0703
    Source: Springer Online Journal Archives 1860-2000
    Topics: Energy, Environment Protection, Nuclear Power Engineering , Medicine
    Notes: Abstract Polychlorinated biphenyls (PCBs), polychlorinated camphenes (PCCs) and isomers of DDT and DDE were the predominant organochlorine (OC) hydrocarbons measured in epontic particulate matter, zooplankton, pelagic and benthic amphipods and liver tissue from an abyssal fish collected in the Arctic Ocean. Chlordane, dieldrin and other cyclodienes and hexachlorocyclohexane (HCH) isomers were present at lower concentrations. Levels on a dry weight basis in plankton of various sizes 〈63 μm to 2 mm were similar to those in epontic particulate matter, but on a lipid weight basis, concentrations in smaller plankton were two to five times higher. Organochlorines in amphipods and liver from the glacial eelpout Lycodes frigidus exceeded levels in zooplankton by up to an order of magnitude. Large benthic lysianassid amphipods (Tmetonyx cicada, Anonyx nugax and Eurythenes gryllus) accumulated higher concentrations on a dry and lipid weight basis than small species (Onisimus spp. and Andaniexis spp.) or the under-ice gammaridean amphipod (Gammarus wilkitzkii). No significant differences in OC levels were measured in benthic amphipods collected at different times. However, concentrations in large zooplankton (〉500 μm) collected in August, dominated by adult copepods and ctenophores, contained concentrations of α-HCH, chlordane isomers and other cyclodienes that were two to four times higher than levels in May. Ratios of α-HCH:γ-HCH (5 to 10) were similar to those in seawater collected simultaneously but there was no difference in ratios in various size categories of planktonic and benthic crustaceans indicating no selective accumulation or metabolic alteration of these isomers. Ratios of cis-chlordane:trans-chlordane concentrations were lower in all sizes of zooplankton (2 to 3) than in shelf amphipods (3 to 6) which corresponded to an increase in the ratio with depth. Higher ratios of DDT:DDE in plankton (2 to 6) than in amphipods (1 to 2) reflects the metabolism of DDT to the more stable DDE isomers in amphipods. Metabolites of trans-chlordane were also measured in plankton and benthic amphipods. Although some OCs are degraded or metabolically transformed, accumulation in lipid-rich tissues results in the highest total concentrations in long-lived large-bodied arctic marine organisms.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00213301
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  • 17
    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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  • 18
    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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  • 19
    Electronic Resource
    Electronic Resource
    Post- and prenatal diagnostic methods for the homocystinurias (1998)
    Springer
    European journal of pediatrics 157 (1998), S. S88 
    Details
    ISSN: 1432-1076
    Keywords: Key words Homocystinuria ; Prenatal ; Diagnosis ; Enzyme ; Metabolite
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Diagnosis of the homozygous homocystinurias can be performed by investigations at the metabolite, enzyme and DNA level. The existence of variant forms due to the wide range of genetic variation may result in only small differences in various parameters between controls and affected subjects. 1. Sulphur amino acid concentrations in plasma, especially total homocysteine, are useful in first line diagnostic investigations. 2. Cystathionine-β-synthase (CBS), methylenetetrahydrofolate reductase (MTHFR) and methylfolate homocysteine methyltransferase (MFMT) can be directly assayed in many tissues including fibroblasts (each) and blood cells (except CBS). Indirect whole cell assays which measure pathway activity dependent on a particular enzyme can provide useful diagnostic information. 3. Direct analysis of mutations is available for CBS, MTHFR and recently also for MFMT deficiencies. However the existence of a larger number of very rare, often private, mutations limits the usefulness of this approach in routine diagnosis. The above diagnostic approaches can generally be applied to prenatal diagnosis. Measurement of methylmalonic acid and other metabolites in amniotic fluid by stable isotope dilution / gas chromatography-mass spectrometry is well established for the methylmalonic acidurias. This method has also been applied to combined homocystinuria/methylmalonic aciduria supported by enzyme assays in cultured cells. Total homocysteine measurement in cell free amniotic fluid is also possible, performed so far in 14 cases with two affected fetuses. The indirect assay of methionine formation from [14C] labelled formate in intact cultured amniotic fluid cells has been for prenatal diagnosis of the remethylation defects.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014311
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  • 20
    Electronic Resource
    Electronic Resource
    Homocysteic and homocysteine sulphinic acid exhibit excitotoxicity in organotypic cultures from rat brain (1998)
    Springer
    European journal of pediatrics 157 (1998), S. S112 
    Details
    ISSN: 1432-1076
    Keywords: Key words Homocystinuria ; Excitotoxicity ; Organotypic cultures ; Xenopus oocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The excitotoxic action of homocysteine and related sulphur-containing metabolites was investigated in organotpyic cultures derived from rat brain cortex and hippocampus by inhibition experiments using antagonists selective for different glutamate receptor subtypes. In addition the direct interaction of these metabolites with glutamate receptors expressed in frog oocytes was tested by conventional two electrode voltage clamp techniques. Conclusion Neurodegeneration and epilepsy observed in homocystinuria may be mediated by l-homocysteic and l-homocysteine sulphinic acid. Both metabolites exhibit excitotoxic potency by interaction with different glutamate receptor subtypes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014291
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