15 resultados para Robert Wilson
em University of Queensland eSpace - Australia
Resumo:
This Article does not have an abstract.
Resumo:
Wilson disease is an autosomal recessive copper transport disorder resulting from defective biliary excretion of copper and subsequent hepatic copper accumulation and liver failure if not treated. The disease is caused by mutations in the ATP7B (WND) gene, which is expressed predominantly in the liver and encodes a copper-transporting P-type ATPase that is structurally and functionally similar to the Menkes protein (MNK), which is defective in the X-linked copper transport disorder Menkes disease. The toxic milk (tx) mouse has a clinical phenotype similar to Wilson disease patients and, recently, the tx mutation within the murine WND homologue (Wnd) of this mouse was identified, establishing it as an animal model for Wilson disease. In this study, cDNA constructs encoding the wild-type (Wnd-wt) and mutant (Wnd-tx) Wilson proteins (Wnd) were generated and expressed in Chinese hamster ovary (CHO) cells. The fx mutation disrupted the copper-induced relocalization of Wnd in CHO cells and abrogated Wnd-mediated copper resistance of transfected CHO cells. In addition, co-localization experiments demonstrated that while Wnd and MNK are located in the trans-Golgi network in basal copper conditions, with elevated copper, these proteins are sorted to different destinations within the same cell, Ultrastructural studies showed that with elevated copper levels, Wnd accumulated in large multivesicular structures resembling late endosomes that may represent a novel compartment for copper transport. The data presented provide further support for a relationship between copper transport activity and the copper-induced relocalization response of mammalian copper ATPases, and an explanation at a molecular level for the observed phenotype of fx mice.
Resumo:
Purpose: The aims of this study are two-fold: first, to analyze intraindividual allometric development of aerobic power of 73 boys followed at annual intervals from 8 to 16 yr, and second, to relate scaled aerobic power with level of habitual physical activity and biological maturity status. Methods: Peak (V) over dot O-2 (treadmill), height, and body mass were measured. Biological maturity was based on age at peak height velocity (PHV) and level of physical activity was based on five assessments between 11 and 15 yr and at 17 yr. Interindividual and intraindividual allometric coefficients were calculated. Multilevel modeling was applied to verify if maturity status and activity explain a significant proportion of peak (V) over dot O-2 after controlling for other explanatory characteristics. Results: At most age levels, interindividual allometry coefficients for body mass exceed k = 0.750. Intraindividual coefficients of peak (V) over dot O-2 by body mass vary widely and range from k' = 0,555 to k' = 1,178. Late maturing boys have smaller k' coefficients than early maturing boys. Conclusion: Peak (V) over dot O-2 is largely explained by body mass, but activity level and its interaction with maturity status contribute independently to peak (V) over dot O-2 even after adjusting for body mass.
Resumo:
The first eighteen months of the Great War witnessed an unprecedented awakening of interest in the Polish Question, when worldwide attention was drawn to the prolonged devastation of the Polish territories. Thereafter, a steady increase in media comment and criticism, highlighting Poland's plight, fostered public indignation at the continual stalling of humanitarian relief efforts for Polish refugees. Such burgeoning popular sentiment focused wider political attention upon a growing movement for recognition of Polish claims to independence. This particularly proved to be the case for Woodrow Wilson and his administration's budding interest in Poland. Subsequently, nowhere did the Polish Question assume a greater role in diplomatic efforts to mediate for peace than in America, and at no time more than during the year preceding the President's hesitant decision to intervene in hostilities.