SACS

SACS
  • 文章类型: Journal Article
    硫化氢(H2S)的催化转化在环境保护和安全生产中起着至关重要的作用。在这次审查中,系统总结了近年来H2S催化转化的理论进展。首先,阐述了H2S催化转化的不同机理。其次,金属表面上H2S催化转化的理论研究,金属化合物,系统综述了单原子催化剂。同时,还综述了为提高催化剂在H2S催化转化中的催化性能而采用的各种策略,主要包括小平面形态控制,掺杂杂原子,金属沉积,有缺陷的工程。最后,提出了H2S催化转化的新方向,并提出了进一步促进H2S转化的潜在策略:包括SAC,双原子催化剂(DAC),单簇催化剂(SCC),沮丧的刘易斯对(FLP),等。本综述可为今后开发用于H2S催化转化的新型催化剂提供见解。
    Catalytic conversion of hydrogen sulfide (H2 S) plays a vital role in environmental protection and safety production. In this review, recent theoretical advances for catalytic conversion of H2 S are systemically summarized. Firstly, different mechanisms of catalytic conversion of H2 S are elucidated. Secondly, theoretical studies of catalytic conversion of H2 S on surfaces of metals, metal compounds, and single-atom catalysts (SACs) are systematically reviewed. In the meantime, various strategies which have been adopted to improve the catalytic performance of catalysts in the catalytic conversion of H2 S are also reviewed, mainly including facet morphology control, doped heteroatoms, metal deposition, and defective engineering. Finally, new directions of catalytic conversion of H2 S are proposed and potential strategies to further promote conversion of H2 S are also suggested: including SACs, double atom catalysts (DACs), single cluster catalysts (SCCs), frustrated Lewis pairs (FLPs), etc. The present comprehensive review can provide an insight for the future development of new catalysts for the catalytic conversion of H2 S.
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  • 文章类型: Case Reports
    SACS基因的突变与CharlevoixSaguenay(ARSACS)的常染色体隐性遗传性痉挛性共济失调有关。它是一种临床和遗传异质性疾病,其特征是缓慢进行性共济失调,痉挛,感觉运动神经病,以及其他表现形式的组合,比如缺乏痉挛,听力损失,和癫痫发作。目前,关于中国患者SACS基因突变的病例报道很少。这里,我们描述了一名35岁的中国患者,在SACS中携带一种新的变异体(c.11486C>T),表现为进行性共济失调和脱髓鞘性周围神经病变.然后我们回顾了22例携带SACS基因突变的中国病例,包括我们的病人.他们都有小脑共济失调步态,并在脑磁共振成像(MRI)上显示小脑萎缩。在这些患者中鉴定出总共28个SACS突变。我们的研究进一步扩展了SACS基因的突变谱,并有助于评估基因型-表型相关性。
    Mutations in the SACS gene have been linked to autosomal recessive spastic ataxia of Charlevoix Saguenay (ARSACS). It is a clinically and genetically heterogeneous disease characterized by slow progressive ataxia, spasticity, sensorimotor neuropathy, and a combination of other manifestations, such as lack of spasticity, hearing loss, and epileptic seizures. Currently, there have been very few case reports regarding the SACS gene mutation in Chinese patients. Here, we describe a 35-year-old Chinese patient carrying a novel variant in SACS (c.11486C>T) presenting with progressive ataxia and demyelinating peripheral neuropathy. We then reviewed 22 Chinese cases carrying SACS gene mutations, including our patient. All of them had a cerebellar ataxia gait and showed cerebellar atrophy on brain magnetic resonance imaging (MRI). A total of 28 SACS mutations were identified in these patients. Our study further expands the mutation spectrum of the SACS gene and contributes to the evaluation of genotype-phenotype correlations.
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