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  • 1
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Factor X (FX) deficiency is a rare bleeding disorder inherited as an autosomal recessive trait. In this study, we investigated the molecular basis of FX deficiency in a Chinese pedigree. The proposita showed a markedly prolonged activated partial thromboplastin time and a mild prolongation of prothrombin time. The levels of FX antigen and FX activity were 58.6% and 2.5%, respectively. Molecular analysis revealed that the proposita was compound heterozygous for two novel mutations: IVS1 + 1G 〉 A and G1185A (Arg347His). The aberrant transcripts from the IVS1 + 1G 〉 A mutant allele were not detected by analyzing the splicing pattern of ectopic transcripts in leukocytes of the patient with nested polymerase chain reaction after reverse transcription. We thus hypothesize that the mRNA molecules originating from the IVS1 + 1G 〉 A mutation were rapidly destroyed in vivo. Site-directed mutagenesis of FX cDNA was used to introduce FXG1185A mutation, and wild-type as well as mutant FX proteins were expressed by transient transfection in HEK 293 cells. Normal FX antigen levels both in the conditioned media of cells expressing the mutant and in cell lysates were detected by an enzyme-linked immunoadsorbent assay. Evaluation of wild-type and mutant coagulant activity demonstrated that the FX molecules carrying the Arg347His mutation have dramatically decreased activity.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  To investigate the molecular defects in two Chinese pedigrees with inherited factor V (FV) deficiency.A 37-year-old male (proband 1) and an 18-month-old boy (proband 2) were diagnosed as inherited coagulation FV deficiency by severely reduced plasma levels of FV activity and antigen. All 25 exons and their flanking sequence of F5 gene were amplified by polymerase chain reaction (PCR) for both probands and the PCR products were directly sequenced. Total RNA was extracted from the peripheral lymphocytes of proband 1 for detecting the changes at mRNA level.The homozygous deletion IVS8 −2A〉G was identified in the F5 gene of proband 1 and complementary DNA (cDNA) analysis revealed the abolishment of the canonical splicing site by the mutation and the activation of the cryptic acceptor site 24 bp upstream instead. The insertion introduced eight additional amino acids (AA) into the FV protein. Two heterozygous mutations of F5 gene were discovered in proband 2. The 2238-9del AG in exon 13 introduced a premature termination code at 689 AA and the substitution of G6410 by T in exon 23 lead to the missense mutation Gly2079Val.Three F5 gene mutations, IVS8 −2A〉G, 2238-9del AG and G6410T, have been identified in two Chinese pedigree with congenital FV deficiency, respectively.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    New York, NY : American Institute of Physics (AIP)
    Physics of Fluids 5 (1993), S. 732-741 
    ISSN: 1089-7666
    Source: AIP Digital Archive
    Topics: Physics
    Notes: This paper discusses the interaction between perpendicular magnetohydrodynamic shocks (S) as well as tangential discontinuities (T). The main conclusions are as follows: (1) The collision between a forward shock (S↘) and a reverse shock (S(downward left arrow)) produces a shock system consisting of a weaker reverse shock, a tangential discontinuity, and a weaker forward shock, namely, S↘S(downward left arrow)→S(downward left arrow)TS↘. (2) The merging of two forward shocks leads to a stronger forward shock, followed by a tangential discontinuity and a reverse wave. For a given βr (the ratio of gas pressure to magnetic pressure) of the background plasma, there exists a critical parameter γc. When the adiabatic index γ〈γc, the reverse wave is a rarefaction wave (R(downward left arrow)). When γ(approximately-greater-than)γc, the reverse wave produced by merging of strong shocks is still a rarefaction wave but the reverse wave becomes a shock when weak shocks merge. (3) The interaction between a forward shock and a tangential discontinuity may form S(downward left arrow)TS↘, R(downward left arrow)TS↘, or TS↘, depending on the strength of the initial forward shock and the property of the initial tangential discontinuity.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Chinese Astronomy and Astrophysics 15 (1991), S. 284-291 
    ISSN: 0275-1062
    Keywords: Sun-magnetic field-coral mass ejection
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Physics
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Chinese Astronomy and Astrophysics 11 (1987), S. 171-178 
    ISSN: 0275-1062
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Physics
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Chinese Astronomy and Astrophysics 15 (1991), S. 292-297 
    ISSN: 0275-1062
    Keywords: The Sun-magnetic field-slow shock
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Physics
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Astrophysics and space science 243 (1996), S. 141-147 
    ISSN: 1572-946X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract This review briefly surveys some recent progress in the numerical simulation study of magnetohydrodynamic (MHD) shock waves in the solar wind.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Astrophysics and space science 83 (1982), S. 189-194 
    ISSN: 1572-946X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract In this paper, a new ideal magnetohydrodynamic (MHD) model is used to examine the dynamical response of the upper solar atmosphere to injection of cold mass from the photosphere akin to a surge. A significant new physical phenomenon is revealed: the formation of an almost stationary loop prominence in the atmosphere as a consequence of the ejected material. Simultaneously with the formation of this new loop, the simulation exhibits MHD waves that propagate outward (i.e., away from the loop) to excite coronal material. It is conjectured that these waves may trigger a class of coronal disturbances.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Solar physics 83 (1983), S. 195-205 
    ISSN: 1573-093X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract This paper considers the structural properties of a sunspot-like magnetic flux tube which lacks perfect axisymmetry. The flux tube is taken to be in static equilibrium with an atmosphere in a uniform gravity. Assuming the departure from axisymmetry to be slight, the equations for the first order non-axisymmetric part of the equilibrium are derived in cylindrical coordinates. These first order equations reduce to a linear second order hyperbolic partial differential equation in the r-z plane. Whereas Cauchy type boundary conditions are appropriate for hyperbolic equations, physical considerations dictate the specification of boundary conditions on a closed surve for our problem of interest. The construction of solutions to this boundary value problem is illustrated with three analytically soluble cases, where the zero-order axisymmetric equilibria are chosen to have magnetic field geometry of different complexity. A physical discussion of the results is given.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Solar physics 84 (1983), S. 83-98 
    ISSN: 1573-093X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract The free energy associated with current sheets formed by displacing magnetic dipoles in a highly conducting medium is discussed. Specific models are illustrated, based on the idea that the free energy of a magnetic field in the solar atmosphere is the energy librated after the field has relaxed to a potential state that preserves the photospheric flux distribution. Previous calculations by other authors based on the consideration of discrete currents were incorrect because the interaction between the atmospheric currents and the highly conducting photosphere was not accounted for properly. It is shown that when this interaction is included, the consideration of discrete currents leads to the same result based on continuous magnetic field consideration.
    Type of Medium: Electronic Resource
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