2篇
您的检索式：作者名="Muhammad Imran Naseer"
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| 1 | 二元金属硫族化合物在能源储存体系中的作用:挑战和可能的解决策略显示文摘二元金属硫族化合物由于其丰富的相界面、高活性位点、优异的导电性以及快速的电化学动力学,与单金属硫族化合物相比具有更加优异的电化学性能.然而,材料的本征离子电导率低,在充放电过程中结构发生反复的团聚与断裂,体积膨胀大等因素导致其电化学性能衰减严重.为了解决上述问题,诸多策略被提出并用于调控二元金属硫族化合物的纳米结构以获得优化的电极材料.但是目前依旧缺乏对二元金属硫族化合物电化学过程的系统认识,也因此限制了其大规模的商业化应用.在这篇综述中,我们不仅重点介绍了基于二元金属硫族化合物电极材料制备的最新研究进展,还通过解释二元金属硫族化合物的电化学动力学,进一步构建和理解了这类材料的构效关系.此外,我们讨论了通过纳米结构化和使用碳材料与三维模板与之形成复合材料的策略来调控和修饰二元金属硫族化合物,并将详细讨论二元金属硫族化合物在超级电容器、金属离子电池、金属空气电池以及碱金属电池等电化学能源储存体系中的工作机理.最后,我们总结了二元金属硫族化合物在发展实际能源器件的过程中面临的主要挑战及可能的解决策略.我们相信,这篇综述将对如何调控二元金属硫族化合物的理化性质以获得优异的电化学器件提供指导性建议. | Yousaf Muhammad Naseer Ufra Ali Imran 李一举 Aftab Waseem Mahmood Asif Mahmood Nasir 高鹏 姜银珠 郭少军 | 2022 | Science China Materials2022，65，3： | 0 |
| 2 | Autosomal recessive cerebellar ataxia with spasticity due to a rare mutation in GBA2 gene in a large consanguineous Saudi family显示文摘The nonlysosomal glucosylceramidase b2(GBA2)gene encode an enzyme that catalyzes the hydrolysis of glucosylceramide to glucose and ceramide.Mutations in the GBA2 gene have been reported to cause hereditary spastic paraplegia,autosomal recessive cerebellar ataxia with spasticity,and Marinescu-Sjogren-Like Syndrome.In this study,we report the clinical features and genetic diagnosis of autosomal recessive cerebellar ataxia with spasticity due to a rare mutation in GBA2 gene in a large consanguineous Saudi family.We included a large consanguineous Saudi family with a presumptive clinical diagnosis of ataxia at King Abdulaziz Medical City in Jeddah,Saudi Arabia.The family included six affected individuals and four unaffected in addition to the parents.Whole exome sequencing(WES)was performed for the probandⅣ-5,and Sanger sequencing was used to confirm the variant in other family members.Segregation study was performed using DNA from the parents and siblings of the proband.Sequence analysis identified a homozygous variant c.2618G>A,p.(Arg873His)in GBA2 gene.The homozygous variant was identified in affected members of the family while the parents and the other four siblings were heterozygous carriers of the variant.One sibling was not available for genetic testing.The variant identified in our patients is classified as pathogenic considering the current evidence of the variant.Autosomal recessive cerebellar ataxia with spasticity is an extremely rare genetic disorder with very few cases reported in the literature.We conclude that the c.2617G>A mutation in GBA2 gene causes the loss of function with abolishment of the enzymatic activity that causes the disease.This report adds further evidence to support the pathogenicity of this variant.The patients had the classical clinical phenotype of cerebellar ataxia and spasticity consistent with previous reports in the literature. | Hussein Algahtani Bader Shirah Ikram Ullah Mohammad H.Al-Qahtani Angham Abdulrahman Abdulkareem Muhammad Imran Naseer | 2021 | Genes & Diseases2021，8，1： | 0 |
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