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1.
Cell surface and secreted proteins provide essential functions for multicellular life. They enter the endoplasmic reticulum (ER) lumen co-translationally, where they mature and fold into their complex three-dimensional structures. The ER is populated with a host of molecular chaperones, associated co-factors, and enzymes that assist and stabilize folded states. Together, they ensure that nascent proteins mature properly or, if this process fails, target them for degradation. BiP, the ER HSP70 chaperone, interacts with unfolded client proteins in a nucleotide-dependent manner, which is tightly regulated by eight DnaJ-type proteins and two nucleotide exchange factors (NEFs), SIL1 and GRP170. Loss of SIL1′s function is the leading cause of Marinesco-Sjögren syndrome (MSS), an autosomal recessive, multisystem disorder. The development of animal models has provided insights into SIL1′s functions and MSS-associated pathologies. This review provides an in-depth update on the current understanding of the molecular mechanisms underlying SIL1′s NEF activity and its role in maintaining ER homeostasis and normal physiology. A precise understanding of the underlying molecular mechanisms associated with the loss of SIL1 may allow for the development of new pharmacological approaches to treat MSS. 相似文献
2.
A. I. D’Souza M. G. Stapelbroek P. N. Dolan P. S. Wijewarnasuriya R. E. DeWames D. S. Smith J. C. Ehlert 《Journal of Electronic Materials》2003,32(7):633-638
The 1/f noise in photovoltaic (PV) molecular-beam epitaxy (MBE)-grown Hg1−xCdxTe double-layer planar heterostructure (DLPH) large-area detectors is a critical noise component with the potential to limit
sensitivity of the cross-track infrared sounder (CrIS) instrument. Therefore, an understanding of the origins and mechanisms
of noise currents in these PV detectors is of great importance. Excess low-frequency noise has been measured on a number of
1000-μm-diameter active-area detectors of varying “quality” (i.e., having a wide range of I-V characteristics at 78 K). The
1/f noise was measured as a function of cut-off wavelength under illuminated conditions. For short-wave infrared (SWIR) detectors
at 98 K, minimal 1/f noise was measured when the total current was dominated by diffusion with white noise spectral density
in the mid-10−15A/Hz1/2 range. For SWIR detectors dominated by other than diffusion current, the ratio, α, of the noise current in unit bandwidth
in(f = 1 Hz, Vd = −60 mV, and Δf = 1 Hz) to dark current Id(Vd = −60 mV) was αSW-d = in/Id ∼ 1 × 10−3. The SWIR detectors measured at 0 mV under illuminated conditions had median αSW-P = in/Iph ∼ 7 × 10−6. For mid-wave infrared (MWIR) detectors, αMW-d = in/Id ∼ 2 × 10−4, due to tunneling current contributions to the 1/f noise. Measurements on forty-nine 1000-μm-diameter MWIR detectors under
illuminated conditions at 98 K and −60 mV bias resulted in αMW-P = in/Iph = 4.16 ± 1.69 × 10−6. A significant point to note is that the photo-induced noise spectra are nearly identical at 0 mV and 100 mV reverse bias,
with a noise-current-to-photocurrent ratio, αMW-P, in the mid 10−6 range. For long-wave infrared (LWIR) detectors measured at 78 K, the ratio, αLW-d = in/Id ∼ 6 × 10−6, for the best performers. The majority of the LWIR detectors exhibited αLW-d on the order of 2 × 10−5. The photo-induced 1/f noise had αLW-P = in/Iph ∼ 5 × 10−6. The value of the noise-current-to-dark-current ratio, α appears to increase with increasing bandgap. It is not clear if
this is due to different current mechanisms impacting 1/f noise performance. Measurements on detectors of different bandgaps
are needed at temperatures where diffusion current is the dominant current. Excess low-frequency noise measurements made as
a function of detector reverse bias indicate 1/f noise may result primarily from the dominant current mechanism at each particular
bias. The 1/f noise was not a direct function of the applied bias. 相似文献
3.
4.
介绍一种基于快速平台测井要求的多通道核能谱测井仪设计方案。复杂可编程逻辑器件的应用使仪器功能增强,规模减小。该系统可对岩性密度长短源距脉冲信号、自然γ能谱脉冲信号进行多道脉冲幅度分析,并对补偿中子测井信号及井下多路常规模拟信号、脉冲信号进行数字化采集。数据传输采用曼彻斯特编码,传输方式可选择20kB/s半双工(兼容Atlas 3508)或下行20kB/s、上行41.66或93.75kB/s全双工方式(兼容Atlas WTS),适应挂接到不同测井系统的需要。 相似文献
5.
介绍了用自然澄清法降低、回收旋涡炉渣中的铜的方法。该方法工艺简单。操作方便,不仅降低冶炼成本,同时提高了经济效益。 相似文献
6.
在每一周期的开始时的速度值V1预设定到传动装置上进行速度预控制,同时,反馈回来的实际值Sj与S1进行比较计算,得出当时偏差值△S,此偏差值△S乘以比例系数Ksp,得到Vk叠加在Vt上,得出传动装置应具有的速度控制信号V,然后V经速度/电压变换和D/A转换向传动系统的速度控制发出控制信号Uα控制执行机构的定位。调节Ksp的值使系统无超调无振荡。 相似文献
7.
Photoacousticspectroscopy (PAS)hasbeenusedtonondestructivelyinvestigatealmostallkindsofsam ples,whetherthesampleiscrystalline ,powderorgel .PAspectr oscopyisacalorimetrictechniquewhichmeasurestheenergyabsorbedandsubsequentheatingofthesamplesduetoanonradiativerelaxationpro cess .ThereforePAScanbeusedtostudyopaque ,highlyreflectiveandscatteringsamples ,whicharedif ficulttostudybyconventionalspectroscopytech niques[1] .Withouttheneedofsamplepreparation ,PASavoidstheriskofsamplealteration ,pre… 相似文献
8.
Jan‐Chan Huang 《应用聚合物科学杂志》2003,90(3):671-680
A modified method is discussed that is based on Farooque and Deshpande's method to obtain polymer–polymer interaction parameters using inverse gas chromatography (IGC) data. In the Farooque and Deshpande method, the ratio of the difference of probe–polymer interaction parameters between two polymers and the probe volume [(χ12 ? χ13)/V1] is used as the abscissa. In the modified method, the ratio [(?2χ12 + ?3χ13)/V1] is used as the abscissa. Experimental data previously reported for a poly(?‐caprolactone)‐polyepichlorohydrin (PCL/PECH) blend and a poly(ethyl acrylate)‐poly(vinyl propionate) (PEA/PVPr) blend are analyzed. It is found that the slopes obtained by the new method had smaller deviations from the theoretical values than the Farooque and Deshpande method. The standard deviations of both slopes and intercepts obtained from the new method are also smaller. Using the new method, the polymer–polymer interaction parameters obtained from the intercept are negative numbers for the PCL/PECH system and very small positive numbers for PEA/PVPr. Explanations are given for the probe and concentration dependency of the polymer–polymer interaction parameters that are generally observed in IGC studies. A new method for selecting the best probe for calculating the interaction parameter is discussed. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 90: 671–680, 2003 相似文献
9.
T. MATTILA-SANDHOLM T. ALI-VEHMAS G. WIRTANEN U. RÖNNER M. SANDHOLM 《International Journal of Food Science & Technology》1991,26(3):325-336
Automated monitoring of the microbiological quality of heat-processed foods by the resazurin reduction test was applied to microtitration plate incubator-fluorimeter technology. The appearance and disappearance of the fluorescing peak of resorufin was monitored on microtitration trays. Pasteurized or ultra-high temperature-treated starch-based soup was used as the model food system. Bacillus subtilis spores (ultra-high temperature treatment) and vegetative cells of Enterococcus faecalis (pasteurization) were inoculated into the soup before the heat treatment at levels which resulted in some survival. The timing of appearance of maximum fluorescence correlated with the number of bacteria in pre-incubated samples. Automated resazurin-reduction fluorimetry was compared with conventional plating, turbidometry and microcolony count by the direct epifluorescent filter technique. The results of the resazurin test correlated well with those of all the other methods tested. Fluorimetry had the advantage that the results could be read within 1–5h and the reproducibility was superior to the other methods. 相似文献
10.
K0.9Li0.1(Ta0.5Nb0.5)O3晶体压电应变系数的测量 总被引:4,自引:2,他引:2
用准静态d_(33)测量仪和干涉法相结合。测量了K_(0.9)Li_(0.1)(Ta_(0.5)Nb_(0.5)Nb_(0.5))O_3晶体的压电应变系数。结果为:d_(33)=86.0,d_(33)=一29.5,d_(15)=112.9×10 ̄(-12)C/N. 相似文献