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  • 1
    Electronic Resource
    Electronic Resource
    Atomic hydrogen-assisted molecular beam epitaxy for the fabrication of multi-quantum-well solar cells (1999)
    [S.l.] : American Institute of Physics (AIP)
    Journal of Applied Physics 86 (1999), S. 5858-5861 
    Details
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The atomic hydrogen-assisted molecular beam epitaxy (H-MBE) technique has been applied to fabricate multi-quantum-well (MQW) based compound semiconductor solar cells. A number of different InGaAs/GaAs MQW structures were inserted within the photon absorption region of a p–i–n GaAs junction diode. The spectral response characteristics of each device with varying number, thickness, and In composition of MQWs were measured and analyzed to investigate the effects of atomic H on the device performance. In the photon wavelength region of 800–1000 nm, the spectral response of H-MBE-grown cells was increased by 〉25% for the GaAs homojunction control cell, and by as much as ∼35% for a 10-period MQW cell (In0.07Ga0.93As: GaAs=50 nm:50 nm) as compared to the conventionally MBE-grown cells with identical layer structures. © 1999 American Institute of Physics.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.371603
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  • 2
    Electronic Resource
    Electronic Resource
    Electrical properties of ion-implanted polyacetylene films (1987)
    [S.l.] : American Institute of Physics (AIP)
    Journal of Applied Physics 61 (1987), S. 5487-5488 
    Details
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: Ion-implantation studies of (CH)x films were carried out with ∼10-keV sodium ions. The temperature dependence of the sheet resistance of the implanted layer exhibited a thermal-activation-type behavior. Its activation energy decreased with increasing the ion dose. A long-term observation of the capacitance-voltage characteristics of an implanted p-n junction showed that a step junction was formed long after implantation as a result of the competition between defects annealing and dopants diffusion. These results ensure that low-energy ion implantation is a useful process for the n-type doping of (CH)x.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.338244
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  • 3
    Electronic Resource
    Electronic Resource
    Accelerated transport and maturation of lysosomal α–galactosidase A in Fabry lymphoblasts by an enzyme inhibitor (1999)
    [s.l.] : Nature America Inc.
    Nature medicine 5 (1999), S. 112-115 
    Details
    ISSN: 1546-170X
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Fabry disease is a disorder of glycosphingolipid metabolism caused by deficiency of lysosomal α–galactosidase A (α–Gal A), resulting in renal failure along with premature myocardial infarction and strokes. No effective treatment of this disorder is available at present. ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/4801
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  • 4
    Electronic Resource
    Electronic Resource
    Slow Evolutionary Rate of GB Virus C/Hepatitis G Virus (1999)
    Springer
    Journal of molecular evolution 48 (1999), S. 383-389 
    Details
    ISSN: 1432-1432
    Keywords: Key words: GBV-C/HGV — GBV-A — Phylogenetic tree — Substitution rate — Divergence time
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Abstract. With the aim of elucidating evolutionary features of GB virus C/hepatitis G virus (GBV-C/HGV), molecular evolutionary analyses were conducted using the entire coding region of this virus. In particular, the rate of nucleotide substitution for this virus was estimated to be less than 9.0 × 10−6 per site per year, which was much slower than those for other RNA viruses. The phylogenetic tree reconstructed for GBV-C/HGV, by using GB virus A (GBV-A) as outgroup, indicated that there were three major clusters (the HG, GB, and Asian types) in GBV-C/HGV, and the divergence between the ancestor of GB- and Asian-type strains and that of HG-type strains first took place more than 7000–10,000 years ago. The slow evolutionary rate for GBV-C/HGV suggested that this virus cannot escape from the immune response of the host by means of producing escape mutants, implying that it may have evolved other systems for persistent infection.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00006482
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  • 5
    Electronic Resource
    Electronic Resource
    GM1-gangliosidosis: Accumulation of ganglioside GM1 in cultured skin fibroblasts and correlation with clinical types (1978)
    Springer
    Human genetics 〈Berlin〉 43 (1978), S. 127-131 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Uptake of radioactivity from 14C-galactose into gangliosides by cultured skin fibroblasts was studied. GM3 was the major ganglioside in control human fibroblasts. An increase of GM1 was demonstrated in GM1-gangliosidosis fibroblasts. The degree of GM1 accumulation was correlated with the clinical types of this disease. The fibroblasts from an infantile-type patient showed a marked increase of GM1. In late-onset types the amount of total gangliosides was only slightly increased, but the distribution of individual gangliosides was definitely abnormal; a relative increase of GM1 was demonstrated in these cases. GM1 β-galactosidase activities were not detectable in either infantile or late-onset cases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293589
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  • 6
    Electronic Resource
    Electronic Resource
    β-Galactosidase-neuraminidase deficiency in adults: Deficiency of a freeze-labile neuraminidase in leukocytes and fibroblasts (1981)
    Springer
    Human genetics 〈Berlin〉 58 (1981), S. 387-389 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary 4-Methylumbelliferyl neuraminidase activity was studied in fibroblasts, leukocytes, and frozen tissues from adult patients with β-galactosidase-neuraminidase deficiency and specific clinical manifestations. This enzyme was almost completely deficient in fibroblasts, but the residual activity was relatively high (20% of the control mean) in the leukocytes from the patients. The frozen liver from one patient showed the enzyme activity as high as controls. This enzyme consisted of two components, freeze-labile and freeze-stable, and it was demonstrated that only the labile enzyme was deficient in fibroblasts and leukocytes. The apparently normal activity of neuraminidase in frozen autopsy tissues of a patient may be explained by the loss of the labile component in control tissues after a long-term freezing. The neuraminidase activity was variable in parents and no definite conclusion was drawn on the hereditary nature of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00282820
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  • 7
    Electronic Resource
    Electronic Resource
    β-Galactosidase deficiency in juvenile and adult patients (1977)
    Springer
    Human genetics 〈Berlin〉 36 (1977), S. 219-229 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Six juvenile and adult patients with progressive neurological diseases and β-galactosidase deficiency were reported. Any diseases known to date were denied. These cases together with ten case reports in the literature were reviewed and were classified into three groups from clinical and biochemical points. Group 1 patients were characterized by progressive ataxia and myoclonus with gargoyle changes and macular cherry-red spots. In this syndrome β-galactosidase activity seems to be secondarily affected by other biochemical defects. A group 2 patient showed similar neurological manifestations without gargoyle changes or macular cherry-red spots. Patients with these clinical features not associated with β-galactosidase deficiency have also been described in the literature. Group 3 patients had progressive pyramidal and extrapyramidal disease without gargoyle changes or macular cherry-red spots. These cases may represent juvenile and adult type GM1-gangliosidosis. Accumulation of GM1 has not yet been demonstrated.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00273261
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  • 8
    Electronic Resource
    Electronic Resource
    β-Galactosidase-deficient human fibroblasts: uptake and processing of the exogenous precursor enzyme expressed by stable transformant COS cells (1990)
    Springer
    Human genetics 〈Berlin〉 85 (1990), S. 505-508 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary COS-1 cells were transfected by electroporation with a cDNA for human acid β-galactosidase cloned in our laboratory and stable transformants expressing the enzyme activity were selected. The precursor form of the enzyme was secreted in large quantities into the culture medium. The fibroblasts from patients with GM1-gangliosidosis or Morquio B disease showed a remarkable increase of enzyme activity, up to the normal level, after culture in this medium for 2 days; the amount of uptake was essentially the same as that for the precursor form in human fibroblasts. After endocytosis, the precursor molecules were processed normally to the mature form and remained as stable as those produced by human fibroblasts. On the other hand, cells from galactosialidosis patients did not show any increase of enzyme activity in a similar experiment. It was concluded that the transformants are useful as the source of precursor proteins for the study of intracellular turnover of enzyme molecules in mutant cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00194225
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  • 9
    Electronic Resource
    Electronic Resource
    Point mutations in the upstream region of the α-galactosidase A gene exon 6 in an atypical variant of Fabry disease (1992)
    Springer
    Human genetics 〈Berlin〉 89 (1992), S. 29-32 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Single point mutations in the upstream region of exon 6 of the α-galactosidase A gene were found in two Japanese cases of the cardiac form of Fabry disease; 301Arg→Gln (902G→A) in a case that has already been published and 279Gln→Glu (835C→G) in a new case. They both expressed markedly low, but significant, amounts of residual activity in COS-1 cells. In contrast, two unrelated cases with classic Fabry disease were found to have different point mutations, which showed a complete loss of enzyme activity in a transient expression assay; 328Gly→Arg (982G→A) in the downstream region of exon 6 in one case and two combined mutations, 66Glu→Gln (196G→C)/112Arg→Cys (334C→T), in exon 2 in the other. We conclude, on the basis of the results recorded in this study and those in previous reports, that the pathogenesis of atypical Fabry disease is closely associated with point mutations in the upstream region of exon 6 of the α-galactosidase A gene.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00207037
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  • 10
    Electronic Resource
    Electronic Resource
    Intracellular processing and maturation of mutant gene products in hereditary β-galactosidase deficiency (β-galactosidosis) (1994)
    Springer
    Human genetics 〈Berlin〉 93 (1994), S. 109-114 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Heterogeneous patterns of biosynthesis, post-translational processing, and degradation were demonstrated for mutant enzymes in three clinical forms of β-galactosidase deficiency (β-galactosidosis): juvenile GM1-gangliosidosis, adult GM1-gangliosidosis, and Morquio B disease. The precursor of the mutant enzyme in adult GM1-gangliosidosis was not phosphorylated, and only a small portion of the gene product reached the lysosomes. The enzyme in Morquio B disease was normally processed and transported to lysosomes, but its catalytic activity was low. A common gene mutation in juvenile GM1-gangliosidosis (R201C) produced an enzyme protein that did not aggregate with protective protein in the lysosome, and was rapidly degraded by thiol proteases. This abnormal turnover was similar to that for the normal but dissociated β-galactosidase in galactosialidosis. Protease inhibitors restored the enzyme acitivity in fibroblasts of this clinical form. A possible therapeutic approach is discussed for this specific type of enzyme deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00210592
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