强流脉冲离子束辐照钨靶温度场演化数值研究

强流脉冲离子束(HIPIB)使材料表面气化产生喷发等离子体从而进行薄膜生长、纳米粉的制备等工作。金属钨的熔点高,热导性好,是耐高热流密度防护层最可能的材料。为了研究金属钨在高热负荷下的响应,建立了HIPIB辐照钨靶的考虑相变的非线性热力学方程,并采用有限元方法数值求解了靶材辐照后的二维温度场演化问题,得到了钨靶温度场的时空演化规律。当离子束流密度为100 A/cm2时,脉冲结束后束流入射中心表层约0.3μm厚的材料熔化,且熔化是从表面开始的,而后因热传导固化;脉冲结束后开始时靶材表面温度急剧下降,而后变得缓慢,温降的梯度在不同时刻均存在峰值,且随时间增加峰值向纵向深度处移动。     

Exome sequencing identifies ACSF3 as a cause of combined malonic and methylmalonic aciduria

We used exome sequencing to identify the genetic basis of combined malonic and methylmalonic aciduria (CMAMMA). We sequenced the exome of an individual with CMAMMA and followed up with sequencing of eight additional affected individuals (cases). This included one individual who was identified and diagnosed by searching an exome database. We identify mutations in ACSF3, encoding a putative methylmalonyl-CoA and malonyl-CoA synthetase as a cause of CMAMMA. We also examined a canine model of CMAMMA, which showed pathogenic mutations in a predicted ACSF3 ortholog. ACSF3 mutant alleles occur with a minor allele frequency of 0.0058 in ～1,000 control individuals, predicting a CMAMMA population incidence of ～1:30,000. ACSF3 deficiency is the first human disorder identified as caused by mutations in a gene encoding a member of the acyl-CoA synthetase family, a diverse group of evolutionarily conserved proteins, and may emerge as one of the more common human metabolic disorders.