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4. Blockchain-Based Ciphertext Policy-Hiding Access Control Scheme

Author

Du, Ruizhong, Zhang, Tianhe, Akan, Ozgur, Editorial Board Member, Bellavista, Paolo, Editorial Board Member, Cao, Jiannong, Editorial Board Member, Coulson, Geoffrey, Editorial Board Member, Dressler, Falko, Editorial Board Member, Ferrari, Domenico, Editorial Board Member, Gerla, Mario, Editorial Board Member, Kobayashi, Hisashi, Editorial Board Member, Palazzo, Sergio, Editorial Board Member, Sahni, Sartaj, Editorial Board Member, Shen, Xuemin, Editorial Board Member, Stan, Mircea, Editorial Board Member, Jia, Xiaohua, Editorial Board Member, Zomaya, Albert Y., Editorial Board Member, Li, Fengjun, editor, Liang, Kaitai, editor, Lin, Zhiqiang, editor, and Katsikas, Sokratis K., editor

Published
2023
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9. Phenotypic and genetic characterization of children with Wilson Disease from Northeast China.

Author

Zhang, Tianhe, Song, Wenliang, and Mao, Zhiqin

Subjects
GENETIC testing, COPPER, GENETIC variation, GENE frequency, LIVER failure
Abstract

Background: Wilson disease (WD) is an autosomal recessive inherited disease caused by ATP7B variants and characterized by copper metabolism defects. However, children with WD are often asymptomatic, making the clinical diagnosis difficult. Therefore, more accurate methods are required for clinical diagnosis. The objective of this study was to highlight the phenotypic and genetic characteristics of children with WD in northeast China. Methods: We retrospectively analyzed the clinical data and gene sequencing results of 65 children with WD from January 1, 2014, to December 31, 2022, at the Shengjing Hospital of China Medical University. All data refer to the time of diagnosis before treatment. Results: The median age at diagnosis was 5 years (range 1.2–15 years). In 50 cases (50/65, 76.9%) patients, routine physical examinations revealed only abnormal liver function. However, they had a significantly negative (p < 0.05) Kayser–Fleischer ring (KF). Children with acute liver failure had significantly increased 24 h urinary copper excretion (p < 0.05). We detected 46 genetic variants of ATP7B, including seven novel variants. The most frequent variant was p.R778L with an allele frequency of 38.7%. Phenotype-genotype correlation analysis suggested that p.R778L was significantly associated with lower serum ceruloplasmin levels and higher zinc levels (p < 0.05). The loss-of-function (LOF) variant was associated with significantly lower albumin levels (p < 0.05). Conclusion: Most children with WD are asymptomatic, which makes early diagnosis of WD difficult. Therefore, clinical and laboratory characteristics as well as genetic testing are essential. p.R778L is the most frequent variant of ATP7B in China and may play an important role in lowering serum ceruloplasmin levels. [ABSTRACT FROM AUTHOR]

Published
2024
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43. Active Recharge Burst and Tonic Spinal Cord Stimulation Engage Different Supraspinal Mechanisms: A Functional Magnetic Resonance Imaging Study in Peripherally Injured Chronic Neuropathic Rats

Author

In Vivo NMR ISI, Circulatory Health, Brain, Meuwissen, Koen P.V., van der Toorn, Annette, Gu, Jianwen Wendy, Zhang, Tianhe C., Dijkhuizen, Rick M., Joosten, Elbert A.J., In Vivo NMR ISI, Circulatory Health, Brain, Meuwissen, Koen P.V., van der Toorn, Annette, Gu, Jianwen Wendy, Zhang, Tianhe C., Dijkhuizen, Rick M., and Joosten, Elbert A.J.

Published
2020

50. Nitrogen-rich salts of 3,6-dinitramino-1,2,4,5-tetrazine: syntheses, structures, and energetic properties.

Author

Li, Haibo, Zhang, Tianhe, Li, Zhimin, Wang, Yanna, Ren, Jie, and Zhang, Tonglai

Subjects
*FURAZANS, *HEAT of combustion, *MOLECULAR structure, *INTERMOLECULAR interactions, *SALTS, COMBUSTION measurement
Abstract

Seven energetic salts of 3,6-dinitramino-1,2,4,5-tetrazine (DNAT), named as (NH4)2(DNAT) (1), (NH2NH3)2(DNAT) (2), (NH3OH)2(DNAT) (3), G2(DNAT) (4) (G = guanidine), (AG)2(DNAT) (5) (AG = aminoguanidine), (DAG)2(DNAT)·H2O (6) (DAG = diaminoguanidine) and (TAG)2(DNAT) (7) (TAG = triaminoguanidine) were synthesized and fully characterized. Molecular structures of 1, 4, 6, and 7 were confirmed by single-crystal X-ray diffraction for the first time, which constituted by the nitrogen-rich cations and DNAT2- through electrostatic and H-bond interactions. The thermal behaviors of 1–7 were also investigated, all of these salts have a higher thermal decomposition temperature than DNAT. Enthalpies of formation of these salts were evaluated according to the measurement results of combustion energies, and the detonation performances were calculated via the EXPLO5 v6.01 program. Compound 7 (P: 31.7 GPa, Dv: 9333 m s−1) exhibits the highest detonation performance among them. Sensitivities toward mechanical stimuli of 1–7 were tested by using BAM standard methods, 4 is insensitive to impact and frication (IF>40 J, FS>360 N). Moreover, the relationship between the sensitivity and intermolecular interactions were analyzed by using Hirshfeld surfaces calculations. All studies demonstrated the salts for potential energetic materials. [ABSTRACT FROM AUTHOR]

Published
2022
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