Role of the HSP70 Co-Chaperone SIL1 in Health and Disease

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.     

1/f noise in large-area Hg<Subscript>1−x</Subscript>Cd<Subscript>x</Subscript>Te photodiodes

The 1/f noise in photovoltaic (PV) molecular-beam epitaxy (MBE)-grown Hg_1−xCd_xTe 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/Hz^1/2 range. For SWIR detectors dominated by other than diffusion current, the ratio, α, of the noise current in unit bandwidth i_n(f = 1 Hz, V_d = −60 mV, and Δf = 1 Hz) to dark current I_d(V_d = −60 mV) was α_SW-d = i_n/I_d ∼ 1 × 10⁻³. The SWIR detectors measured at 0 mV under illuminated conditions had median α_SW-P = i_n/I_ph ∼ 7 × 10⁻⁶. For mid-wave infrared (MWIR) detectors, α_MW-d = i_n/I_d ∼ 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 α_MW-P = i_n/I_ph = 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, α_MW-P, in the mid 10⁻⁶ range. For long-wave infrared (LWIR) detectors measured at 78 K, the ratio, α_LW-d = i_n/I_d ∼ 6 × 10⁻⁶, for the best performers. The majority of the LWIR detectors exhibited α_LW-d on the order of 2 × 10⁻⁵. The photo-induced 1/f noise had α_LW-P = i_n/I_ph ∼ 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.     

基于Intranet/Internet的大坝安全监测信息查询系统

大坝安全监测信息的网上发布可以让人们了解各种实时、动态信息，文 中主要介绍了在Intranet/Internet下采用ASP(active server pages)结合VBScript，JavaS cript及ActiveX技术进行大坝安全监测信息查询系统开发的方法及步骤，并结合实际系统的 开发加以说明。     

TCP稳态流中慢启动序列对吞吐量性能的影响

在计算TCP稳态数据流吞吐量时一般都忽略了由于分组丢失而形成的各个慢启动阶段。在考虑TCP稳态数据流中慢启动序列的情况下,对Padhye的经典TCP稳态数据流模型进行了扩展。通过将Padhye的模型与扩展后的模型作比较,证明了在使用Padhye的模型作为多媒体流量控制模型时忽略超时后慢启动阶段的可行性和正确性。     

堇青石基玻璃陶瓷的制备与展望

介绍了制备堇青石基玻璃陶瓷的三种方法：熔融法、烧结法和溶胶-凝胶法，综述了玻璃组成、添加剂和成核剂对堇青石基玻璃陶瓷的烧结及性能的影响，并对其未来的发展进行了展望。     

引进意大利连续纺丝机车间空调节能设计

介绍了某化纤厂引进意大利十二台粘胶连续纺丝机车间与以往的纺丝车间相比,空调设计中的节能途径,体现了既满足工艺要求,又降低生产成本的设计目的。     

法国产CT4型横拼双金属专用真空电子束焊机原理与构造

试述法国TECHMETA公司产的CT4型横拼双金属专用真空电子束焊机原理与构造，其中主要阐述电子枪控制电路板的原理。     

A broad line n.m.r. study of oriented poly(vinylidene fluoride)

Broadline nuclear magnetic resonance measurements have been carried out for the proton resonance in oriented poly(vinylidene fluoride). The spectra in general show two component lines with distinctly different line widths. The broad component shows a high degree of molecular orientation and can be satisfactorily assigned to the crystalline regions of the polymer. The narrow component corresponds to an oriented non crystalline phase which is sufficiently constrained to allow motion about the chain axis only. An appreciable decrease in the value of the rigid mass fraction was observed in both of the samples over the temperature range examined. Our calculations indicate that this could play an important role in the temperature dependence of the pyroelectric response of this material.     

含氯取代基的聚间苯二甲酰间苯二胺的合成与表征

以 2 ,5 -二氯对苯二甲酰氯作为第三单体 ,将其与间苯二甲酰氯、间苯二胺在N ,N -二甲基乙酰胺中进行低温溶液共缩聚反应 ,合成了含氯取代基的聚间苯二甲酰间苯二胺。研究了单体摩尔浓度、反应初始温度、叔胺添加剂种类、第三单体用量等对共聚物相对分子质量的影响 ,并用红外光谱、热重分析等方法对共聚物进行了表征。     

心电图信号的频域分析

提出了一种简便有效的、由心电图（ECG）曲线提取QRS-T复合波的新方法，从而获得P波（当房颤未发生时）或f波（当房颤时）；利用功率谱、自相关、互相关等信号处理技术，对它进行了处理和分析，特别是，比较了未房颤时和发生房颤时的相关信息的显著差别。