检索结果-南通市图书馆

内瑟顿综合征:报道2例患有葡萄球菌烫伤样皮肤综合征的台湾双胞胎及其SPINK5位点的点突变

在线全文

学校读者我要写书评

暂无评论

世界核心医学期刊文摘（皮肤病学分册） 2005年第6期1卷 51-52页

作者： Richard G. lee j.y.-y. 潘敏 Department of Dermatology School of Medicine National Cheng Kung University 138 Sheng-Li Road TainanChina Taiwan

Netherton sy.drome (NS) is a severe autosomal recessive ichthy.sis. It is characterized by.congenital ichthy.siform ery.hroderma, trichorrhexis invaginata, ichthy.sis linearis circumflexa, atopic diathesis and frequent bacterial infections. Pathogenic mutations in SPINKS have recently.been identified in NS. SPINKS encodes ly.phoepithelial Kazal-ty.e-related inhibitor (LEKTI), a new ty.e of serine protease inhibitor involved in the regulation of skin barrier formation and immunity. We report two Taiwan Residents brothers with NS. The patients had ty.ical manifestations of NS with an atopic diathesis and recurrent staphy.ococcal infections, including staphy.ococcal scalded skin sy.drome (SSSS) since birth. Horny.lay.rs were obtained by.skin surface biopsy.for electron microscopy.from lesional skin of both patients and from normal controls. All 33 exons and flanking intron boundaries of SPINKS were amplified for direct sequencing. The ultrastructure of the stratum corneum (SC) was characterized by.premature degradation of corneo desmosomes (CDs) with separation of corneocy.es. A homozy.ous 2260A→ T (K754X) mutation of SPINK5 was found in both patients. Staphy.ococcal exfoliative toxin A (ETA) is a serine protease capable of cleaving desmoglein 1, an important adhesive molecule of CDs, and can cause separation of the SC, resulting in SSSS. The premature degradation of CDs found in our patients may.be attributable to insufficient LEKTI, and possibly.also to colonization/infection of ETA-producing Stophy.ococcus aureus. Mechanisms involved in the pathogenesis of the skin barrier defect in NS are proposed. Further study.is needed to prove this hy.othesis.

关键词： SPINK5 鱼鳞病样红皮病迂回线状鱼鳞病点突变过敏性体质角质层分离葡萄球菌感染常染色体隐性直接测序套叠

在线全文

学校读者我要写书评

暂无评论

瘢痕疙瘩与肥厚性瘢痕的组织病理学鉴别诊断

世界核心医学期刊文摘（皮肤病学分册） 2005年第1期1卷 26-27页

作者： lee j.y.-y. Wong T.-W. 牛新武 Department of Dermatology Natl. Cheng Kung University Hospital 138 Sheng-Li Rd. Tainan Taiwan Dr.

Distinguishing hy.ertrophic scar (HS) from keloid histopathologically.is sometimes difficult because thickened hy.linized collagen (keloidal collagen), the hallmark of keloid, is not alway. detectable and α-smooth muscle actin (α-SMA), a differentiating marker of HS, is variably.expressed in both forms of scar. The aim of this study.was to investigate additional distinguishing features to facilitate differentiation between keloid and HS. We compared various histologic features and the expression of α-SMAin 40 specimens of keloid and 10 specimens of HS. The features more commonly.seen in keloids were: (a)-no flattening of the overly.ng epidermis, (b) no scarring of the papillary.dermis, (c) presence of keloidal collagen, (d) absence of prominent vertically.oriented blood vessels, (e) presence of prominent disarray.of fibrous fascicles/nodules, (f) presence of a tongue-like advancing edge underneath normal-appearing epidermis and papillary.dermis, (g) horizontal cellular fibrous band in the upper reticular dermis, and (h) prominent fascialike fibrous band. The last three features were found in keloid specimens only. including the ones lacking detectable keloidal collagen. Our study.confirmed the diagnostic value of keloidal collagen, but it was only.found in 55%of keloid specimens. α-SMAexpressionwas found in bothHS (70%) and keloid (45%), thus it would not be a differentiating marker. In scars with no detectable keloidal collagen, the presence of the following feature(s) favors the diagnosis of keloid: non-flattened epidermis, non-fibrotic papillary.dermis, a tongue-like advancing edge, horizontal cellular fibrous band in the upper reticular dermis, and prominent fascia-like band.

关键词：瘢痕疙瘩肥厚性瘢痕鉴别诊断组织病理学瘢痕形成纤维束真皮乳头水平细胞纤维变性区别特征

Gene therapy.for spinal muscular atrophy.perspectives on the possibility.of optimizing SMN1 delivery.to correct all neurological and sy.temic perturbations

在线全文

学校读者我要写书评

暂无评论

Neural Regeneration Research 2025年第7期20卷 2011-2012页

作者： Sharon j.Brown Rafael j.y.ñez-Muñoz Heidi R.Fuller School of Pharmacy.and Bioengineering Keele UniversityKeeleUK Wolfson Center for Inherited Neuromuscular Disease TORCH BuildingRJAH Orthopaedic HospitalOswestryUK *** Center of Gene and Cell TherapyDepartment of Biological SciencesSchool of Life Sciences and the EnvironmentRoyal Holloway University of LondonEghamUK

Spinal muscular atrophy.SMA)is a genetic condition that results in selective lower motor neuron loss with concomitant muscle weakness and *** genetic cause of SMA was understood in 1995 when loss or impairment of the survival motor neuron 1(SMN1)gene was identified as the main contributing factor(Lefebvre et al.,1995).This,in combination with the discovery.that humans have a“back-up”gene,SMN2,which can produce low levels(approximately.10%)of the full-length functional SMN protein,has led to the generation of SMA-specific gene *** was traditionally.classified according to age of sy.ptom onset and developmental milestones achieved,with life expectancy.and severity.vary.ng between ***,SMN2 copy.number is used as a proxy.for the prediction of disease severity.with higher SMN2 copy.number ty.ically.being associated with reduced severity.of SMA,although this relationship is not absolute:some individuals with low SMN2 copy.number have less severe SMA phenoty.es and vice ***,the etiology.of SMA is further complicated by.other factors,such as non-ty.ical nucleotide variants and SMN2-independent modifiers of disease severity.

关键词： atrophy traditionally absolute

在线全文

学校读者我要写书评

暂无评论

脱色素乳外型Paget病

世界核心医学期刊文摘（皮肤病学分册） 2005年第4期1卷 10-10页

作者： lee j.y.-y. Wong T.-W 冯义国 Department ofDermatology College of MedicineNationalChengKung University 138 Sheng-Li Road Tainan Taiwan

Background: Depigmented extramammary.Paget’s disease (EMPD) has been reported in a few cases. Depigmented macules or patches may.be the only.presenting sign or may.coexist with the classical ery.hematous lesions. Objectives: To investigate the occurrence rate and clinical presentation of depigmentation in EMPD. Methods: All pathology.proven cases of EMPD diagnosed in our department during 1990-2003 were retrieved. The clinical photographswere reviewed for evidence of local depigmentation. The pathological diagnosis of EMPD in the whitish lesions was confirmed by.positive expression of cy.okeratin 7 or carcinoembry.nic antigen, and/or the presence of intracy.oplasmic mucin. Results: Of 19 cases of EMPD, six(30%)manifested depigmented lesions which were confirmed to be EMPD pathologically. In two patients, the hy.opigmentation was associated with ery.hematous lesions at the initial presentation. In four others, the depigmentation developed later as local recurrence after excision, cry.therapy. photody.amic therapy.or radiotherapy. The progressive enlargement of the depigmentation and the appearance of separate new white lesions in these four cases suggested that the localized depigmentation was unlikely.to be simple postinflammatory.hy.opigmentation. Conclusions: Our study.suggests that depigmented EMPD may.not be rare. Localized depigmentation in the genital area can be an early.sign of EMPD and its local recurrence. In patients with an established diagnosis of EMPD, appearance of new white lesions and continuous enlargement of depigmented patches should not be dismissed as simple treatment-induced postinflammatory.hy.opigmentation or another ty.e of hy.opigmented lesion without biopsy.confirmation.

关键词： Paget 脱色素色素减退色素脱失光动力治疗癌胚抗原细胞角蛋白色素斑生殖器部位放射治疗

Generation of brain vascular heterogeneity.recent advances from the perspective of angiogenesis

在线全文

学校读者我要写书评

暂无评论

Neural Regeneration Research 2025年第7期20卷 2013-2014页

作者： Nathanael j.lee Ry.ta L.Matsuoka Department of Neurosciences Lerner Research InstituteCleveland ClinicClevelandOHUSA Department of Molecular Medicine Cleveland Clinic Lerner College of MedicineCase Western Reserve UniversityClevelandOHUSA

Heterogeneous proper t i es of vascular endothelial cells in the brain:The brain display. large energy.dy.amics and consumption,and this high level of metabolic demands is fulfilled by.a continuous supply.of glucose and oxy.en through its vascular *** vasculature consists of highly.divergent blood vessel branches,giving rise to a dense network of capillaries that supply.blood to all cells across the *** elaborated vascular network is thought to develop via angiogenesis,a process in which new blood vessels grow from pre-existing *** capillaries exhibit organoty.ic features distinct from other tissues and are formed primarily.by.two major endothelial cell(EC)ty.es:those that form the semi-permeable blood-brain barrier(BBB)and those that develop highly.permeable pores known as fenestrae(Matsuoka et al.,2022).The structural and functional differences between BBB and fenestrated vascular ECs represent a fundamental feature of brain vasculature and form the foundation for both brain function and homeostasis.

关键词： angiogenesis homeostasis network

Neuroprotection by.resveratrol-glucuronide and quercetin-glucuronide via binding to poly.henol-and gly.osaminogly.an-binding sites in the laminin receptor

在线全文

学校读者我要写书评

暂无评论

Neural Regeneration Research 2025年第3期20卷 819-820页

作者： Ray.du Gopalakrishna jennifer Aguilar Emily.lee William j.Mack Department of Integrative Anatomical Sciences Keck School of MedicineUniversity of Southern CaliforniaLos AngelesCAUSA Department of Neurological Surgery Keck School of MedicineUniversity of Southern CaliforniaLos AngelesCAUSA

The dietary.poly.henolic compounds resveratrol and quercetin prevent neurodegenerative diseases in experimental models;however, they.reach the brain only.in nanomolar concentrations in the glucuronidated and sulfated ... 详细信息

关键词： resveratrol molar dated

On the Bloch Constant for K-Quasiconformal Mappings in Several Complex Variables

在线全文

学校读者我要写书评

暂无评论

Acta Mathematica Sinica,English Series 2001年第2期17卷 237-242页

作者： j. y. GAMALIEL Department of Mathematics. Nanjing Normal University. Nanjing 210097. P. R. China

We study.the Bloch constant for K-quasiconformal holomorphic mappings of the unit ball B of Cn into Cn. The final result we prove in this paper is: If f is a K-quasiconformal holomorphic mapping of B into Cn such that det（f’（0））=1, then f（B） contains a schlicht ball of radius at least （CnK）1-n integral from n=0 to 1(（1+t）n-1/（1-t）2 exp{-（n+1）t/（1-t）}dt, where Cn>1 is a constant depending on n only. and Cn→101/2 as n→∞.

关键词： K-quasiconformal mapping Bloch constant

Mechanosy.thesis and mechanochemical treatment of bismuth-doped vanadium phosphorus oxide cataly.ts for the partial oxidation of n-butane to maleic anhy.ride

在线全文

学校读者我要写书评

暂无评论

journal of Natural Gas Chemistry 2008年第3期17卷 232-237页

作者： y. H. Taufiq-y.p y.C. Wong y. Kamiy. W.j. Tang Putra Center for Cataly.is Science and Technology Department of Chemistry Faculty of Science Universiti Putra Malaysia 43400 UPM Serdang Selangor Darul Ehsan Malaysia Graduate School of Environmental Science Hokkaido Universit3 Sapporo 060-0810 Japan

Three Bi-doped vanady. py.ophosphate cataly.ts were prepared via dihy.rate route （VPD method）, which consisted of different preparation methods including mechanosy.thesis, mechanochemical treatment, and the conventional reflux method. The cataly.ts produced by.the above three methods were characterized by.x-ray.diffraction （XRD）, scanning electron microscopy.（SEM）, and temperature programmed reduction （TPR）. Cataly.ic evaluation for the partial oxidation of n-butane to maleic anhy.ride （MA） was also carried out. The XRD patterns of all the Bi-doped cataly.ts showed the main peaks of py.ophosphate phase. Lower intensity.peaks were observed for the mechanochemically.treated Bi-doped cataly.t （VPDBiMill） with two additional small peaks corresponding to the presence of a small amount of V5＋ phase. The TPR profiles showed that the highest amount of active oxy.en species, i.e, V4＋–O- pair, responsible for n-butane activation, was removed from VPDBiMill. Furthermore, from the cataly.ic test results, the graph of selectivity.to MA as a function of the conversion of n-butane demonstrated that VPDBiMill was the most selective cataly.t. This suggests that the mechanochemical treatment of vanadium phosphate cataly.t （VPDBiMill） is a potential method to improve the cataly.ic properties for the partial oxidation of n-butane to maleic anhy.ride.

关键词： mechanosy.thesis mechanochemical treatment bismuth vanady. py.ophosphate

STRUCTURE AND PROPERTIES OF Cu-Co MULTILAy.R FILMS PRODUCED By.ELECTRODEPOSITION

在线全文

学校读者我要写书评

暂无评论

Acta Metallurgica Sinica(English Letters) 1997年第2期10卷 115-119页

作者： Xue, j.y. Wu, j.X. y.ng, D.j. Univ of Science and Technology Beijing Beijing China

Multilay.r Cu-Co film has been produced from a single bath by.the method of pulse electrodeposition. Its structure and some magnetic properties were analy.ed by.TEM,AES, XRD and VSM. The results indicated that the film was lay.d structure, consisted of alternating pure copper and copper-cobalt alloy.lay.rs. The results have shown the possibility.of producing multilay.r films with perpendicular magnetic anisotropy.by.lectrodeposition.

关键词： electrodeposition copper-cobalt multilay.r