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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.
The design and operation principle of existing portable mechanization means and technological features of their application in assembling joints in plating are investigated; structural and technological features of tooling and assembly equipment are described, the physiology of labour in assembling plating joints is analysed, including static and dynamic loading of the hands when carrying over, holding and application of assembling means on different levels in relation to the height of the worker body, the maximum allowable weight and size parameters of portable mechanization means for assembling plating joints are determined and the results of development of new specialized equipment for mechanization of assembling of joints’ pneumohydraulic operating mode are described.  相似文献   
3.
For decades, numerous artificial muscles have been proposed in order to implement beneficial features of biological muscles into robotics. Unfortunately, traditional artificial muscles experienced difficulties in imitating properties of the biological muscles due to mechanical and control issues. Recently, twisted and coiled polymer actuators (TCP) have been shown to produce large mechanical power via thermal stimulations and strong linearity. In this paper, a high-performance TCP thermally cycled by electrical heating and forced convective liquid cooling is designed and associated control algorithms are presented. We elaborate the model of the TCP that is simple, yet provides insight into how the electrical heating and the forced convective liquid cooling contribute to the TCP actuation. The proposed model is verified by experimental studies. Based on the proposed model, we design a feedforward–feedback controller and switching laws, which actively control the TCP in both the heating and cooling cycles. Furthermore, we extend our control methodology to agonist–antagonist TCPs. From the experimental studies, the proposed method is shown to be effective in both single TCP and antagonistic TCPs.  相似文献   
4.
Energy metabolism is a basic and general process, by which the body acquires and uses energy to maintain normal function, and taurine plays a vital role in energy metabolism. Taurine deficiency may cause a weak energy metabolism and energy metabolism dysfunction. Taurine biosynthetic ability is limited, and its supplementation in the diet can strengthen energy metabolism in muscle performance, cardiac function, liver activity, and adipose tissue. Combining taurine with other drugs may have a superior effect in energy metabolism. In many metabolic disorders, taurine, or the combination of taurine with other drugs, also functions as a repair treatment for damaged tissues, and acts as a promoter for the balance of energy metabolism. The present study discusses the potential roles of taurine in energy metabolism.  相似文献   
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脂肪干细胞因具有体外快速增殖、能向多谱系细胞分化、低免疫原性等一系列成体干细胞的生物学特性,使其在细胞治疗领域有很大的应用潜力。本文介绍了脂肪干细胞的生物学特性,及其在放射性骨骼肌损伤中的应用研究进展。  相似文献   
7.
为从肌纤维特性方面探究牦牛3 个部位肌肉品质的差异,本研究以牦牛背最长肌、腰大肌和半膜肌为研究对象,比较了其剪切力、肌节长度、肉色、蒸煮损失率和pH值的差异,采用酶组织化学染色和实时荧光定量聚合酶链式反应法表征了其肌纤维特性,并分析了每个品质指标与肌纤维之间的相关性。结果表明:3 个部位肌肉的品质指标差异明显,其中腰大肌有较高的pH值和a*值,嫩度较好,而背最长肌保水性较好;3 个部位的肌纤维特性差异显著(P<0.05),腰大肌具有较高比例的I型肌纤维(50.8%)和较高的MYH7 mRNA相对表达量,肌纤维直径显著低于背最长肌和半膜肌(P<0.05);I型肌纤维数目比和肉品质密切相关,其与剪切力呈负相关,与肌节长度和蒸煮损失率呈正相关;I型肌纤维面积比与L*值呈显著负相关(P<0.05)。结论:可以通过提高肌肉中I型肌纤维的比例来改善牦牛肉品质。  相似文献   
8.
动力手套是一种腕部康复训练装置,同时具有辅助的助力功能。康复训练是手腕进行自主康复的一种重要的辅助治疗手段,传统的康复训练装置大多采用电机驱动的刚性外骨骼结构,在运动时急剧变化的冲击力易造成关节二次伤害,同时较大的质量增加了身体的额外负担。基于气动人工肌肉设计并制作了一款柔性可穿戴动力手套装置,能够实现腕关节屈/伸、内收/外展两自由度运动,集康复训练与腕部助力于一体,轻量化柔顺的外骨骼结构提高了其便携性及舒适性。通过嵌入薄膜压力传感器能够感知手掌与物体之间的抓取力,自动实现腕关节助力功能并有效地保护腕部。对腕部进行穿戴静力分析求解手套装置的性能需求参数,并测试气动肌腱的关键指标及手套的整体性能,测试结果表明所设计的动力手套能够满足腕部康复训练要求并具有辅助的助力功能。  相似文献   
9.
采用液相色谱- 串联质谱(LC-MS/MS)法测定动物肌肉组织中氯霉素,甲砜霉素和氟苯尼考三种药物的残留量。添加氘代氯霉素(d5- 氯霉素)作为内标,碱化乙酸乙酯提取,正己烷去除脂肪,省去固相萃取步骤直接上机定量。选择电喷雾离子源(ESI),负离子扫描多反应监测(MRM)模式,并优化了质谱参数,使仪器的灵敏度达到最高。氯霉素、甲砜霉素和氟苯尼考的检出限均达到0.01 μ g/kg。空白样品中添加氯霉素、甲砜霉素和氟苯尼考水平为0.02~1 μ g/kg 时,三种药品的线性相关系数均大于0.9990。氯霉素平均回收率为92.8%~97.0%,甲砜霉素平均回收率为93.5%~96.6%,氟苯尼考平均回收率为91.2%~95.9%。  相似文献   
10.
The aim of this study is to evaluate the ability of physical training in the maintenance of muscle strength in rats with HFD (high-fat diet) after OVX (ovariectomy). Eighty Wistar rats are at eight weeks of age and weight 200 g which divided into 8 groups (n = 10) and treated for 12 weeks: GA: OVX + ND (normal diet), GB: OVX + ND + training, GC: sham + ND, GD: sham + ND + training, GE: OVX + HFD, GF: OVX + HFD + training, GG: sham + HFD and GH: sham + HFD + training. HFD consists of standard ration for rats with addition of 30% lipids. In training groups, physical training five training/week was conducted on a treadmill with adaptation period of three weeks up to 18 m/s for one hour, training were performed for 12 weeks. The sedentary animals remained in individual box. To analyze the effects of training and diet, tensile strength tests of the gastrocnemius muscles were conducted: the speed of 0.1 mm/min. Analysis of variance was performed to compare groups. The mean (SD) obtained for the maximum load (N) were: GA 57.77 (6.89), GB 62.74 (5.07), GC 49.45 (6.06), GD 59.42 (5.26) and GE 55.58 (4.72), GF 62.50 (4.56), GG 58.35 (4.54) and GH 56.67 (5.87), respectively. There were no differences for maximum load between surgeries (p = 0.004) and between treatments (p = 0.000). Differences were found also for the relationship surgery diet treatment (p = 0.007). For the variable stiffness (N/mm), there were not statistically significant differences: GA 5.03 (0.72), GB 5.08 (1.09), GC 5.17 (0.53), GD 5.35 (0.80), GE 5.52 (1.20), GF 5.36 (1.07), GG 4.83 (1.03) and GH 5.40 (0.73). For the toughness (N/mm), there were differences between treatments (p = 0.010) and the ratio diet treatment (p = 0.024): GA 455.00 (107.21), GB 541.96 (126.80), GC 394.97 (84.67), GD 566.90 (157.07); GE 424.63 (113.03), GF 478.07 (106.03); GG 517.44 (98.65?  相似文献   
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