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转塔式锚泊系统的动力分析 总被引:5,自引:2,他引:3
采用时域分析方法计算转塔式锚泊系统的动力响应问题,并对系泊在水深350m的常规的转塔式锚泊系统进行了分析研究.计算结果与响应模型试验进行了比较,两者吻合较好.在此基础上,对系泊在水深400m和1200m的常规及具有浮筒的转塔式锚泊系统在各种环境条件作用下的运动和受力进行了分析研究,提出了对深海转塔式锚泊系统应注意的问题,这对深海石油开采装置的研究具有一定的实用意义 相似文献
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Vonk WI de Bie P Wichers CG van den Berghe PV van der Plaats R Berger R Wijmenga C Klomp LW van de Sluis B 《Cellular and molecular life sciences : CMLS》2012,69(1):149-163
Menkes disease (MD) is an X-linked recessive disorder characterized by copper deficiency resulting in a diminished function
of copper-dependent enzymes. Most MD patients die in early childhood, although mild forms of MD have also been described.
A diversity of mutations in the gene encoding of the Golgi-resident copper-transporting P1B-type ATPase ATP7A underlies MD. To elucidate the molecular consequences of the ATP7A mutations, various mutations in ATP7A associated with distinct phenotypes of MD (L873R, C1000R, N1304S, and A1362D) were analyzed in detail. All mutants studied
displayed changes in protein expression and intracellular localization parallel to a dramatic decline in their copper-transporting
capacity compared to ATP7A the wild-type. We restored these observed defects in ATP7A mutant proteins by culturing the cells
at 30°C, which improves the quality of protein folding, similar to that which as has recently has been demonstrated for misfolded
ATP7B, a copper transporter homologous to ATP7A. Further, the effect of the canine copper toxicosis protein COMMD1 on ATP7A
function was examined as COMMD1 has been shown to regulate the proteolysis of ATP7B proteins. Interestingly, in addition to
adjusted growth temperature, binding of COMMD1 partially restored the expression, subcellular localization, and copper-exporting
activities of the ATP7A mutants. However, no effect of pharmacological chaperones was observed. Together, the presented data
might provide a new direction for developing therapies to improve the residual exporting activity of unstable ATP7A mutant
proteins, and suggests a potential role for COMMD1 in this process. 相似文献
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