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.     

Biotransformation of l‐menthol by twelve isolates of soil‐borne plant pathogenic fungi (Rhizoctonia solani) and classification of fungi

The microbial transformation of l‐menthol ( 1 ) was investigated by using 12 isolates of soil‐borne plant pathogenic fungi, Rhizoctonia solani (AG‐1‐IA Rs24, Joichi‐2, RRG97‐1; AG‐1‐IB TR22, R147, 110.4; AG‐1‐IC F‐1, F‐4, P‐1; AG‐1‐ID RCP‐1, RCP‐3, and RCP‐7) as a biocatalyst. Rhizoctonia solani F‐1, F‐4 and P‐1 showed 89.7–99.9% yields of converted product from 1 , RCP‐1, RCP‐3, and RCP‐7 26.0–26.9% and the other isolates 0.1–12.0%. In the cases of F‐1, F‐4 and P‐1, substrate 1 was converted to (?)‐(1S,3R,4S,6S)‐6‐hydroxymenthol ( 2 ), (?)‐(1S,3R,4S)‐1‐hydroxymenthol ( 3 ) and (+)‐(1S,3R,4R,6S)‐6,8‐dihydroxymenthol ( 4 ), which was a new compound. Substrate 1 was converted to 2 and/or 3 by RRG97‐1, 110.4, RCP‐1, RCP‐3 and RCP‐7. The structures of the metabolic products were elucidated on the basis of their spectral data. In addition, metabolic pathways of the biotransformation of 1 by Rhizoctonia solani are discussed. Finally, from the main component analysis and the differences in the yields of converted product from 1 , the 12 isolates of Rhizoctonia solani were divided into three groups based on an analysis of the metabolites. Copyright © 2003 Society of Chemical Industry     

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.     

Comparison of inward and outward grown Pt modified aluminide diffusion coatings on a Ni based single crystal superalloy

Two commercial Pt modified aluminide coatings (RT22 and MDC150L) on the same single crystal Ni-based superalloy (CMSX-4) were studied by: scanning electron microscopy; transmission electron microscopy; energy dispersive X-ray spectrometry; and gravimetry. The RT22 coating is an inward grown coating (high activity), while MDC150L is produced by outward growth (low activity). Samples were oxidised in still laboratory air at 1050 °C for various times up to 2000 h. It was found that the outward grown coating produced a slower growing oxide that was more spallation resistant. Several possible reasons for this were identified including: coating purity; coating surface topography; and ductile to brittle transition temperature. The microstructural differences between the two coatings in the as-coated condition were investigated and the development of their microstructure during heat treatment was described. A model for coating growth during heat treatment was proposed.     

R22替代制冷剂的生产控制评价

本文详细介绍了R2 2替代制冷剂的生产过程控制和整机可靠性评价 ,并介绍了一些具体做法。     

新型高k栅介质材料研究进展

随着半导体技术的不断发展，MOSFET（metal-oxide-semiconductor field effect transistor）的特征尺寸不断缩小，栅介质等效氧化物厚度已小至nm数量级。这时电子的直接隧穿效应将非常显著，将严重影响器件的稳定性和可靠性。因此需要寻找新型高k介质材料，能够在保持和增大栅极电容的同时，使介质层仍保持足够的物理厚度来限制隧穿效应的影响。本文综述了研究高k栅介质材料的意义；MOS栅介质的要求；主要新型高k栅介质材料的最新研究动态；展望了高k介质材料今后发展的主要趋势和需要解决的问题。     

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。     

45钢表面超音速电弧喷涂Ti-Al涂层的组织与性能研究

采用自行开发研制的超音速电弧喷设备，在45钢表面制成了钛铝合金复合涂层。并利用金相显微镜、X射线衍射仪、扫描电镜、电子探针，对涂层的成分、相结构、显微组织及其结合强度、显微硬度进行了研究。结果表明，利用超音速电弧喷涂设备，可以在45钢表面形成 度高、孔隙率低，结合强度较好和硬度高的Ti-Al合金涂层。     

基于MSP430F11X的低功耗温度记录仪

本文详细介绍了基于MSP430F11X的大容量低功耗温度记录仪的工作原理及软硬件设计方法,并给出了实际应用的硬件电路和程序源代码。