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        A novel NPHS2 mutation (c.865A > G) identified in a Chinese family with steroid-resistant nephrotic syndrome alters subcellular localization of nephrin

        Wu Na,Zhu Yingchuan,Jiang Wenhao,Song Yue,Yin Lan,Lu Yilu,Tao Dachang,Liu Yunqiang,Ma Yongxin 한국유전학회 2022 Genes & Genomics Vol.44 No.5

        Background: NPHS2 is the causative gene of nephrotic syndrome type 2 (MIM 600995) which often clinically manifests as steroid-resistant nephrotic syndrome (SRNS). The NPHS2 gene encodes a slit diaphragm (SD) associated protein podocin. Objective: This study reported a novel disease-causing mutation of NPHS2 in a Chinese family with SRNS. We also investigated the pathogenic mechanism of the variants in this family. Method: A Chinese family with SRNS was recruited. Whole exome sequencing was performed to screen for disease-causing mutation. Sanger sequencing was used to confirm the results. In vitro functional experiments including immunoblotting, co-immunoprecipitation and double immunofluorescence staining were performed to explore the pathogenic mechanisms of mutations. Results: In this family, compound heterozygous mutations of NPHS2 (c.467dupT and c.865A > G) were identified and segregated with the disease. The maternal c.865A > G was a novel variant, leading to amino acid substitution (p.K289E). In vitro functional assays indicated that c.467dupT (p.L156FfsX11) mutant lost interaction with nephrin. Both K289E and L156FfsX11 mutants showed sharply diminished plasma membrane localization. Furthermore, abnormal distribution of podocin mutants also altered the cell membrane localization of nephrin. Conclusion: We reported a family with SRNS caused by compound heterozygous mutations of NPHS2 (c.467dupT and c.865A > G). c.865A > G (p.K289E) in NPHS2 was a novel causative variant associated with SRNS. Both variants in this family not only affected the normal cell membrane localization of podocin, but also altered the cell membrane localization of nephrin which is the major architectural protein of SD.

      • KCI등재

        Adaptive Digital Predictive Peak Current Control Algorithm for Buck Converters

        Yu Zhang,Yiming Zhang,Xuhong Wang,Wenhao Zhu 전력전자학회 2019 JOURNAL OF POWER ELECTRONICS Vol.19 No.3

        Digital current control techniques are an attractive option for DC-DC converters. In this paper, a digital predictive peak current control algorithm is presented for buck converters that allows the inductor current to track the reference current in two switching cycles. This control algorithm predicts the inductor current in a future period by sampling the input voltage, output voltage and inductor current of the current period, which overcomes the problem of hardware periodic delay. Under the premise of ensuring the stability of the system, the response speed is greatly improved. A real-time parameter identification method is also proposed to obtain the precision coefficient of the control algorithm when the inductance is changed. The combination of the two algorithms achieves adaptive tracking of the peak inductor current. The performance of the proposed algorithms is verified using simulations and experimental results. In addition, its performance is compared with that of a conventional proportional-integral (PI) algorithm.

      • SCIESCOPUSKCI등재

        Adaptive Digital Predictive Peak Current Control Algorithm for Buck Converters

        Zhang, Yu,Zhang, Yiming,Wang, Xuhong,Zhu, Wenhao The Korean Institute of Power Electronics 2019 JOURNAL OF POWER ELECTRONICS Vol.19 No.3

        Digital current control techniques are an attractive option for DC-DC converters. In this paper, a digital predictive peak current control algorithm is presented for buck converters that allows the inductor current to track the reference current in two switching cycles. This control algorithm predicts the inductor current in a future period by sampling the input voltage, output voltage and inductor current of the current period, which overcomes the problem of hardware periodic delay. Under the premise of ensuring the stability of the system, the response speed is greatly improved. A real-time parameter identification method is also proposed to obtain the precision coefficient of the control algorithm when the inductance is changed. The combination of the two algorithms achieves adaptive tracking of the peak inductor current. The performance of the proposed algorithms is verified using simulations and experimental results. In addition, its performance is compared with that of a conventional proportional-integral (PI) algorithm.

      • KCI등재

        Influence of Water Molecules on Polarization Behavior and Time–Frequency Dielectric Properties of Cellulose Insulation

        Wen Hao,Cheng Lin,Jiang Yi,Zhu Taiyun,Chen Zhong,Dai Xize,Gao Chenyu 대한전기학회 2021 Journal of Electrical Engineering & Technology Vol.16 No.3

        Oil-immersed power transformer plays an important role in power transmission and transformation engineering. Moisture is one of the important factors to determine its insulation performance. Molecular simulation technique is used to study the mechanism of water molecules on the micro polarization behavior of cellulose insulation materials. The number of hydrogen bonds, solubility parameters, total dipole moment and polarizability are analyzed. These characteristics explain the action mechanism of water molecules on cellulose insulation. At the same time, the time domain and frequency dielectric response of cellulose insulation materials with diff erent moisture content are explored. The dielectric modulus spectrum is introduced to replace the traditional frequency dielectric spectroscopy to characterize the micro relaxation process of cellulose insulation under diff erent moisture content. Finally, the characteristic parameters are extracted from the polarization, depolarization current and dielectric modulus spectrum curves to evaluate the moisture content of cellulose insulation materials. The results show that the number of hydrogen bonds, binding energy density, solubility parameter, total dipole moment and polarizability increase with the increase of water content. It should be noted that dielectric modulus spectroscopy can eff ectively characterize the relaxation behavior of cellulose insulation materials. The moisture content of cellulose insulation can be quantitatively evaluated by DC conductivity, insulation resistance, imaginary part relaxation peak of dielectric modulus and integral value of real part and imaginary part of dielectric modulus.

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