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Papular acantholytic dyskeratosis of the anogenital area with novel ATP2C1 gene mutations

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Chinese Medical Journal 2021年第12期134卷 1508-1510页

作者： Xue-Min Xiao Yi-Qun Jiang Wei Tian cheng-rang li Department of Dermatology The Union HospitalFujian Medical UniversityFuzhouFujian 350001China Institute of Dermatology Jiangsu Key Laboratory of Molecular Biology for Skin Diseases and Sexually Transmitted InfectionsChinese Academy of Medical Sciences and Peking Union Medical CollegeNanjingJiangsu 210042China

To the Editor:Focal acantholytic dyskeratosis(FAD)is used to describe a group of diseases with diverse clinical manifestations but characteristic histological structures.In 1984,the first case of“papular acantholytic dyskeratosis(PAD)of the vulvocrural area”was reported in a 23-year-old female with pathological manifestations of FAD and the researchers believed that it might be a new independent disease.[1]Recently,the relationship between PAD and Hailey-Hailey disease(HHD)as well as Darier disease(DD)is receiving increasing attention from scholars.We performed gene mutation screening in two patients with sporadic PAD and explored the nature of PAD.

关键词： diseases kerato sis

Zebrafish Model of Hereditary Pigmentary Disorders

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International Journal of Dermatology and Venereology 2019年第4期2卷 216-220页

作者： Wen-Rui li cheng-rang li lin lin Department of Dermatology Hospital for Skin Diseases (Institute of Dermatology) Chinese Academy of Medical Sciences and Peking Union Medical College Nanjing Jiangsu 210042 China

Introduction Pigmentary disorders are a heterogeneous group of hereditary or acquired disorders characterized by varying degrees of hyperpigmentation and/or hypopigmentation.Pigmentary disorders originate from abnormalities in melanocyte development,defects in melanin synthesis,or changes in melanosome transfer.1-2 Several skin pigmentation disorders and diseases are heritable,including dyschromatosis universalis hereditaria(DUH),Dowling-Degos disease(DDD),and albinism.To elucidate the pathogenesis of these heritable pigmentary disorders,a cross-species approach has been widely applied and has resulted in a detailed understanding of melanocyte physiology and pathophysiology.

关键词： disorders diseases pathogenesis

NCSTN Gene Silencing Inhibits the Retinoic Acid Signaling Pathway in Human Immortalized Keratinocytes

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International Journal of Dermatology and Venereology 2021年第1期4卷 26-31页

作者： Ying-Da Wu Qiu-Xia Mao Yuan-Yuan Zhang Ping cheng Wen-Rui li Yan-Yan He Hao-Xiang Xu Bao-Xi Wang cheng-rang li Department of Dermatology Hospital for Skin Diseases(Institute of Dermatology)Chinese Academy of Medical Sciences and Peking Union Medical CollegeNanjingJiangsu 210042China Department of Dermatology Jiangyin Hospital of Traditional Chinese MedicineJiangyin Hospital Affiliated to Nanjing University of Chinese MedicineJiangyinJiangsu 214400China Jiangsu Key Laboratory of Molecular Biology for Skin Diseases and STIs NanjingJiangsu 210042China Department of Dermatology Plastic Surgery HospitalChinese Academy of Medical Sciences and Peking Union Medical College Plastic Surgery HospitalBeijing 100144China

Objective:Acne inversa is a multifactorial chronic debilitating disease.Genetic factors are involved in 40%of patients,especially the nicastrin(NCSTN)gene.However,the role of the mutated NCSTN gene in the pathogenesis of acne inversa remains unclear.Retinoic acid is recommends to treat moderate to severe acne inversa,therefor we conduct this in vitro research to study the association between NCSTN gene mutation and the retinoic acid signaling pathway in human immortalized skin keratinocyte(HaCaT)cells.Methods:HaCaT cells were infected with a lentivirus-mediated short hairpin RNA(shRNA)expression plasmid specifically targeting the NCSTN gene.Real-time polymerase chain reaction(PCR)and Western blotting were used to detect the interference efficiency of NCSTN.RNA sequencing was used to detect differential genes in the NSCTN-deficient HaCaT cells.Based on bioinformatics analysis and clinical treatment data,the retinoic acid signal pathway was selected for screening.Quantitative PCR was used to verify the changes in the expressions of retinoic acid signaling pathway-related receptors and molecules in the HaCaT cell line after NCSTN silencing.The Student t test and one-way analysis of variance were used to evaluate intergroup differences.Results:Sequencing showed that the NCSTN-shRNA lentiviral recombinant expression plasmid was successfully constructed.After lentivirus infection of HaCaT cells,real-time PCR results showed significantly reduced NCSTN mRNA expression in the interference group compared with the negative control group,and the interference efficiency was 75.0%.Western blotting showed that the inhibition rate of NCSTN protein expression in the shRNA group was 71.7%.RNA sequencing revealed significant differential expression of some genes,and changes in signaling pathways.Compared with the control group,the group with the silenced NCSTN showed significantly decreased expression of retinoic acid receptors(RARα:F=23.482,RARβ:F=603.241,RXRα:F=69.689,and RARRES1:F=167.482,and all Plifer

关键词： acne inversa NCSTN gene retinoic acid signaling pathway

Targeted treatments of psoriasis vulgaris recommended by guidelines

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International Journal of Dermatology and Venereology 2018年第3期1卷 141-150页

作者： Wan-Lu Zhang Ying-Da Wu Ping cheng cheng-rang li Institute of Dermatology Chinese Academy of Medical Sciences and Peking Union Medical CollegeNanjingJiangsu 210042China

Introduction Psoriasis is a common chronic recurrent inflammatory skin disease with most common clinical form of chronic plaque type (90%).For moderate-to-severe psoriasis vulgaris patients,who were inadequately responding to other synthetic therapies,targeting therapeutic drugs may have unexpected effects.And with the deepening understanding of pathogenesis,the targeted therapy for psoriasis is increasingly diverse and accurate.Biologic agents can block T cell activation,antagonise T cell secretion of tumor necrosis factor alpha (TNF-α),interleukin (IL)-17,IL-23,and other cytokines,and then effectively block the immune inflammatory response.

关键词： Targeted treatments psoriasis vulgaris guidelines

Dowling-Degos disease:current research progress

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International Journal of Dermatology and Venereology 2018年第4期1卷 208-211页

作者： Ying-Da Wu Wen-Rui li Wan-Lu Zhang Ping cheng cheng-rang li Department of Dermatology Hospital for Skin Diseases (Institute of Dermatology) Chinese Academy of Medical Sciences and Peking Union Medical College Nanjing Jiangsu 210042 China

Introduction Dowling-Degos disease (DDD) was first described as a benign form of acanthosis nigricans by Dowling and Freudenthal in 1938, then referred to as'dermatose reticulée des plis'by Degos and Ossipowski in 1954, and was first named DDD by Wilson-Jones and Grice in 1978(1)The disease has been reported worldwide and affects both genders equally. As an autosomal dominant pigment disorder, it usually occurs in post-pubertal individuals, and is seldom seen in children(2)However, a Chinese newborn with DDD was reported in 2008(3)In this review, we summarize features of DDD, emphasizing advances in genetics research and looking to the future for further understanding of its etiology and the development of therapeutic methods.

关键词： Dowling-Degos disease acanthosis nigricans

Disseminated pyoderma gangrenosum with oral involvement:successful treatment with systemic steroids and cyclosporine

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International Journal of Dermatology and Venereology 2018年第4期1卷 246-248页

作者： Wen-Rui li Bao-Shu Sun Nan Sheng Si-Jia Zhao Wan-Lu Zhang lin lin cheng-rang li Department of Dermatology Hospital for Skin Diseases (Institute of Dermatology) Chinese Academy of Medical Sciences and Peking Union Medical College Nanjing Jiangsu 210042 China Department of Dermatology First People's Hospital of Tancheng Linyi Shandong 276199 China

Introduction Pyoderma gangrenosum (PG) is a chronic skin disease characterized by progressive lesions that present as painful suppurative ulcers and tend to recur. The pathogenesis of the disease is incompletely under-stood;however, 50%to 70%of affected persons have an associated concomitant systemic disease such as inflammatory bowel disease (IBD) (1)Any site of the body can be affected;however, involvement of the oropharynx is rare. Here, we present a patient with disseminated PG with oropharyngeal involvement.

关键词： Pyoderma gangrenosum chronic skin disease

Mathematical Model,Numerical Simulation and Convergence Analysis of a Semiconductor Device Problem with Heat and Magnetic Influences

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Acta Mathematicae Applicatae Sinica 2024年第2期40卷 302-319页

作者： Chang-feng li Yi-rang YUAN Huai-ling SONG School of Economics Shandong UniversityJinan 250100China Institute of Mathematics Shandong UniversityJinan 250100China College of Mathematics and Econometrics Hunan UniversityChangsha 410082China

In this paper,the authors discuss a three-dimensional problem of the semiconductor device type involved its mathematical description,numerical simulation and theoretical analysis.Two important factors,heat and magnetic influences are involved.The mathematical model is formulated by four nonlinear partial differential equations(PDEs),determining four major physical variables.The influences of magnetic fields are supposed to be weak,and the strength is parallel to the z-axis.The elliptic equation is treated by a block-centered method,and the law of conservation is preserved.The computational accuracy is improved one order.Other equations are convection-dominated,thus are approximated by upwind block-centered differences.Upwind difference can eliminate numerical dispersion and nonphysical oscillation.The diffusion is approximated by the block-centered difference,while the convection term is treated by upwind approximation.Furthermore,the unknowns and adjoint functions are computed at the same time.These characters play important roles in numerical computations of conductor device problems.Using the theories of priori analysis such as energy estimates,the principle of duality and mathematical inductions,an optimal estimates result is obtained.Then a composite numerical method is shown for solving this problem.

关键词： three-dimensional conductor device problem with heat and magnetic influences upwind blockcentered differences elemental conservation of mass numerical analysis

Recent progress in functional modification and crosslinking of bioprosthetic heart valves

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Regenerative Biomaterials 2024年第1期3卷 1-16页

作者： cheng Zheng li Yang Yunbing Wang National Engineering Research Center for Biomaterials Sichuan UniversityChengdu 610064China

Valvular heart disease(VHD),clinically manifested as stenosis and regurgitation of native heart valve,is one of the most prevalent cardiovascular diseases with high mortality.Heart valve replacement surgery has been recognized as golden standard for the treatment of VHD.Owing to the clinical application of transcatheter heart valve replacement technic and the excellent hemodynamic performance of bioprosthetic heart valves(BHVs),implantation of BHVs has been increasing over recent years and gradually became the preferred choice for the treatment of VHD.However,BHVs might fail within 10-15 years due to structural valvular degeneration(SVD),which was greatly associated with drawbacks of glutaraldehyde crosslinked BHVs,including cytotoxicity,calcification,component degradation,mechanical failure,thrombosis and immune response.To prolong the service life of BHVs,much effort has been devoted to overcoming the drawbacks of BHVs and reducing the risk of SVD.In this review,we summarized and analyzed the research and progress on:(i)modification strategies based on glutaraldehyde crosslinked BHVs and(ii)nonglutaraldehyde crosslinking strategies for BHVs.

关键词： bioprosthetic heart valves modification crosslinking anticalcification antithrombosis

Integration of urchin-like MnCo_(2)O_(4)@C core–shell nanowire arrays within porous copper current collector for superior performance li-ion battery anodes

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Rare Metals 2024年第2期43卷 599-611页

作者： Xue-Ru Zhang Qiong Wu Yong Zhang Xi-Fei li Ting Xie Yu-cheng Wu Instrumental Analysis Center Hefei University of TechnologyHefei230009China Key Laboratory of Advanced Functional Materials and Devices of Anhui Province Hefei230009China School of Materials Science and Engineering Hefei University of TechnologyHefei230009China Institute of Advanced Electrochemical Energy Xi’an University of TechnologyXi’an710048China

Spinel MnCo_(2)O_(4) is a promising energy storage candidate as anode materials in lithium-ion batteries owing to synergistic effects of two intrinsic solid-state redox couples.However,low conductivity,poor rate capacity and rapid capacity fading have seriously impaired its practical applications.To overcome the inferiorities,urchin-like MnCo_(2)O_(4)@C core–shell nanowire arrays have been fabricated directly within a porous copper current collector via a facile hydrothermal method followed by a chemical vapor deposition carbonization process.In a typical nanowire,the core is composed of interconnected MnCo_(2)O_(4)nanoparticles and the shell shows as a thin amorphous carbon layer.The integrated MnCo_(2)O_(4)@C/Cu structure could act as working anodes without using additives or polymer binders.While MnCo_(2)O_(4)@C/Cu possesses slightly longer li-ion insertion/desertion pathway than that of MnCo_(2)O_(4)/Cu,the carbon shell could effectively prevent the pulverization of MnCo_(2)O_(4) and lower down charge transfer resistance and actively participate in li-ion cycles.The rearrangement of carbon atoms during lithiation/delithiation cycling could inhibit the formation of passive solid electrolyte interphase films.As a result,the MnCo_(2)O_(4)@C/Cu electrode presents superior rate capacity(600 mAh·g^(−1) at 1 A·g^(−1)) and better stability(797 mAh·g^(−1) after 200 cycles at 100 mA·g^(−1)).The excellent reversible li ion storage capacity,cycling stability and rate capacity endow MnCo_(2)O_(4)@C/Cu great potential as stable and high output integrated anode materials in li-ion batteries.

关键词： MnCo_(2)O_(4)@C Urchin-like nanowire arrays Core-shell Integrated anode materials lithium-ion batteries