Novel KCND3 Variant Underlying Nonprogressive Congenital Ataxia or SCA19/22 Disrupt KV4.3 Protein Expression and K+ Currents with Variable Effects on Channel Properties

KCND3 encodes the voltage-gated potassium channel K_V4.3 that is highly expressed in the cerebellum, where it regulates dendritic excitability and calcium influx. Loss-of-function K_V4.3 mutations have been associated with dominant spinocerebellar ataxia (SCA19/22). By targeted NGS sequencing, we identified two novel KCND3 missense variants of the K_V4.3 channel: p.S347W identified in a patient with adult-onset pure cerebellar syndrome and p.W359G detected in a child with congenital nonprogressive ataxia. Neuroimaging showed mild cerebellar atrophy in both patients. We performed a two-electrode voltage-clamp recording of K_V4.3 currents in Xenopus oocytes: both the p.G345V (previously reported in a SCA19/22 family) and p.S347W mutants exhibited reduced peak currents by 50%, while no K+ current was detectable for the p.W359G mutant. We assessed the effect of the mutations on channel gating by measuring steady-state voltage-dependent activation and inactivation properties: no significant alterations were detected in p.G345V and p.S347W disease-associated variants, compared to controls. K_V4.3 expression studies in HEK293T cells showed 53% (p.G345V), 45% (p.S347W) and 75% (p.W359G) reductions in mutant protein levels compared with the wildtype. The present study broadens the spectrum of the known phenotypes and identifies additional variants for KCND3-related disorders, outlining the importance of SCA gene screening in early-onset and congenital ataxia.     

脉冲激光与电弧布置方式对铝合金焊接熔滴过渡与焊缝形貌的影响

以2219铝合金为基板，研究了不同脉冲激光-电弧布置方式下的熔滴过渡与焊缝形貌特征，分析了熔深增加的机理. 结果表明，当脉冲激光照射母材时，脉冲激光主要提供对母材的热输入，母材温度的增加有助于促进熔滴铺展，稳定熔滴过渡过程；当脉冲激光照射熔滴缩颈时，主要提供对熔滴的力输入，蒸发反力的作用下形成"一脉一滴"，显著提高熔滴过渡频率与熔滴飞行速度，增加了熔滴对熔池的冲击力，熔深增加；当脉冲激光交替的照射熔池和熔滴时，一方面能够对母材进行加热，有助于熔滴的铺展，另一方面能够提高熔滴过渡频率，提高焊缝的均匀性.     

超厚板TC4钛合金电子束焊接接头应力腐蚀敏感性

针对100 mm超厚板TC4钛合金电子束焊接接头，采用慢应变速率拉伸方法评价接头在人造海水中的应力腐蚀敏感性，分析接头的显微组织和断口形貌，对接头的腐蚀机制进行研究. 结果表明，室温条件下应变速率为ε=1×10-6 s-1时，母材在海水中未表现出应力腐蚀敏感性；焊缝上部、中部和下部具有轻微应力腐蚀敏感性. 焊缝在海水中发生阳极溶解，产生氢吸附，导致裂纹的萌生. 同时氢扩散诱导α'相界及α'相内发生位错塞积，进而使裂纹在更低的应力水平下发生扩展.     

0Cr17Ni4Cu4Nb传感器断裂原因分析

某力学传感器在出厂检验流程中发生断裂失效,为了确定失效原因,防止此类失效事件再次发生,实验对该传感器的力学性能、金相组织和断口形貌进行了分析,确定了断裂原因。结果表明,传感器的失效模式为氢致延迟开裂,材料中氢含量偏高和开裂位置机加工质量较差是导致传感器发生氢致开裂的主要原因。结合失效原因,提出了改进措施。     

Mg-5Y-3Sm-0.8Ca-0.5Sb合金的高温蠕变行为

采用蠕变实验和理论计算,研究了时效态Mg-5Y-3Sm-0. 8Ca-0. 5Sb合金在250～300℃和50～70 Pa条件下的蠕变行为。结果表明,该合金具有良好的抗高温蠕变性能,其在300℃和70 MPa下的稳态蠕变速率为3. 28×10-8s-1。在50～70 MPa的应力下,当温度由250℃升到300℃时,应力指数由2. 30变为3. 22;在250～300℃的温度下,当应力由50 MPa升到70 MPa时,蠕变激活能由65. 4 kJ/mol变为81. 0 kJ/mol。     

Improved Corrosion Resistance of Selective Laser Melted Ti–5Cu Alloy Using Atomized Ti–5Cu Powder

The different types of metal powder used for selective laser melting(SLM) process would cause distinct corrosion behavior due to the uniformity of the obtained microstructure.The SLM-produced Ti–5Cu alloy using atomized Ti–5Cu metal powder(SLMed Ti–5Cu) in this work reveals a relatively uniform microstructure with overwhelming acicular α/α′ phase and shows great advantages on corrosion resistance compared with the SLM-produced Ti–5Cu alloy using the mixture powder(SLMedM Ti–5Cu).The effect of the micro-galvanic cells decreases due to the undetectable Ti_2Cu phase in the microstructure of the SLMed Ti–5Cu.An apparent passivation behavior was observed for SLMed Ti–5Cu instead of severe pitting phenomenon for the SLMed-M Ti–5Cu.The charge transfer resistance of SLMed Ti–5Cu in this work is 10.09 ± 2.63 MΩ cm~2, which is significantly higher than that of SLMed-M Ti–5Cu(4.76 MΩ cm~2).The above result indicates the atomized Ti–5Cu powder plays an important role in the formation of the uniform microstructure of SLMed product, thereby enhancing its corrosion resistance in Hank's solution at 37 ℃.     

耐蚀合金中TiN析出机制

摘要：TiN颗粒尺寸及其分布对耐蚀合金性能有明显的影响，因此有必要对TiN在铸坯中的分布及其析出行为进行研究。采用扫描电镜(SEM)、金相显微镜（OM）观察了TiN夹杂物在铸坯中的分布、尺寸及其形貌；基于热力学和动力学理论分析了耐蚀合金铸坯中TiN夹杂物的析出时机及其尺寸，结合试验结果和理论计算明确了TiN夹杂物在凝固后铸坯中的位置和尺寸与析出时机的关系，为控制TiN夹杂物提供理论指导。结果表明，冶炼过程中析出的TiN夹杂物尺寸较大，在凝固过程中被枝晶吞没，位于铸坯枝晶内和等轴晶内；微观偏析计算结果表明，在凝固分数为0.55时，TiN开始析出，最开始析出TiN夹杂物的逐渐长大，长大后的TiN易于被二次枝晶吞没，最终位于铸坯中的枝晶间和等轴晶内，后期析出的TiN则在枝晶间和等轴晶间。固相中析出的TiN夹杂物长大较慢，尺寸细小，最终位于奥氏体晶界。     

两种3D打印用雾化Ti-6Al-4V粉末的对比研究

本研究分别利用水冷铜坩埚真空感应熔炼气雾化(VIGA-CC)和等离子旋转电极(PREP)两种技术制备出球形Ti-6Al-4V合金粉末,作者利用SEM、同步辐射CT扫描-三维重建和氩气含量测试等分析手段对不同粒径的Ti-6Al-4V合金粉末的孔洞缺陷和氩气含量、硬度值进行了表征。实验结果表明, VIGA-CC粉末粒度分布宽,细粉收得率较多,粉末粒度分布在40~180 μm之间, PREP粉末的粒度分布较窄,主要集中在110~180 μm之间；金属粉末内部的孔隙率、气体含量和孔尺寸随着粉末粒度的增大而增大,且同一粒径范围内VIGA-CC粉末的气孔概率多于PREP粉末；随着粉末粒径减小,粉末截面组织逐渐细化,其硬度值逐渐升高,整体上VIGA-CC粉末硬度值高于PREP粉末。     

Oxidation‐led decomposition of hexagonal boron nitride coatings on alloy substrates at 900 °C: Ti‐6Al‐4V

Hexagonal boron nitride (h‐BN) coatings on Ti‐6Al‐4V substrates undergo complete decomposition in air at 900 °C. This fate is similar to that of this ceramic material on chromia‐former alloys, and unlike that of a mass of powder treated in isolation. As the ceramic and alloy oxidize concurrently, outwardly diffusing aluminum (III) ions but not the predominant titanium (IV) ions react with the boron trioxide that forms around the h‐BN basal plane peripheries. Resultant aluminum borate is incorporated into the growing scale and the boron trioxide diffusion barrier is depleted. By this mechanism, the oxidation of h‐BN is maintained at an enhanced rate, until both this material and its oxide completely decompose. Liberated nitrogen from the oxidation of h‐BN can enter the underlying scale as a randomly distributed solute in rutile solid solution. The post‐coating oxide‐atmosphere interface comprises elongated aluminum borate crystallites protruding through at the boundaries between 3–5 at% nitrogen‐doped rutile grains. It differs significantly from that of oxidized, uncoated Ti‐6Al‐4V, which is occupied by a thin α‐alumina layer atop rutile. This interface does not change with an additional 72 h of heat‐treatment.     

Corrosion behavior of under‐, peak‐, and over‐aged 6063 alloy: A comparative study

The mechanical property of age‐hardenable Al‐alloys is governed by the state of ageing, which determines the microstructure and consequently, their corrosion behavior which is a vital aspect for a number of applications. This article presents a comparative assessment of corrosion behavior of under‐, peak‐ and over‐aged Al‐Mg‐Si alloy. Corrosion characteristics have been determined via immersion tests in 0.1 M ortho‐phosphoric acid solution and intergranular corrosion (IGC) tests. Corroded surfaces are examined by field emission scanning electron micrographs‐energy dispersive spectroscopy and 3D optical profilometer. The obtained results reveal that the corrosion rate at a specific immersion time as well as the depth of IGC increases in the order for under‐, peak‐, and over‐aged states. Irrespective of the state of ageing, corrosion loss increases linearly but the rate of corrosion decreases rapidly with increasing immersion time. The dominant mode of corrosion in under‐aged alloy is identified as localized pitting, while peak‐aged is highly susceptible to IGC in contrast extensive pitting corrosion is observed for over‐aged alloy. The observed differences in corrosion behavior are explained considering characteristics of precipitates. Formation of β (Mg₂Si) in case of over‐aged alloy and presence of inclusions like AlFeMnSi particles are found to accelerate pitting corrosion.