• 【研究技术变得简单:自身免疫性水疱病的小鼠模型。】 复制标题 收藏 收藏
    DOI:10.1016/j.jid.2016.11.003 复制DOI
    作者列表:Pollmann R,Eming R
    BACKGROUND & AIMS: :Autoimmune blistering diseases are examples of autoantibody-mediated, organ-specific autoimmune disorders. Based on a genetic susceptibility, such as a strong HLA-class II association, as yet unknown triggering factors induce the formation of circulating and tissue-bound autoantibodies that are mainly directed against adhesion structures of the skin and mucous membranes. Compared with other autoimmune diseases, especially systemic disorders, the pathogenicity of autoimmune blistering diseases is relatively well described. Several animal models of autoimmune blistering diseases have been established that helped to uncover the immunological and molecular mechanisms underlying the blistering phenotypes. Each in vivo model focuses on specific aspects of the autoimmune cascade, from loss of immunological tolerance on the level of T and B cells to the pathogenic effects of autoantibodies upon binding to their target autoantigen. We discuss current mouse models of autoimmune blistering diseases, including models of pemphigus vulgaris, bullous pemphigoid, epidermolysis bullosa acquisita, and dermatitis herpetiformis.
    背景与目标: 自身免疫性水疱病是自身抗体介导的器官特异性自身免疫性疾病的例子。基于遗传易感性,例如强烈的HLA-II类关联,目前尚未发现的触发因素诱导循环和组织结合的自身抗体的形成,这些抗体主要针对皮肤和粘膜的黏附结构。与其他自身免疫性疾病,尤其是全身性疾病相比,自身免疫性水疱病的致病性相对较好地描述。已经建立了几种自身免疫性水疱疾病的动物模型,这些模型有助于揭示水疱表型的免疫学和分子机制。每个体内模型都专注于自身免疫级联反应的特定方面,从T细胞和B细胞水平的免疫耐受性丧失到自身抗体与靶自身抗原结合后的致病作用。我们讨论了自身免疫性水疱疾病的当前小鼠模型,包括寻常性天疱疮,大疱性天疱疮,表皮松解性大疱性水疱病和疱疹样皮炎的模型。
  • 【表皮基底膜:其分子组织和水泡紊乱。】 复制标题 收藏 收藏
    DOI:10.1080/03008200600584157 复制DOI
    作者列表:Masunaga T
    BACKGROUND & AIMS: :The epidermal basement membrane is a specialized structure localized between the epidermis and the dermis. Recent studies have elucidated the biological roles of the basement membrane and its pathophysiological involvement in bullous diseases. To understand the functions of the basement membrane, it is essential to have clear and precise information regarding the ultrastructural molecular organization of the epidermal basement membrane. Immunoelectron microscopy is a powerful technique and the only method available for the clarification of the ultrastructural localization or orientation of molecules. This review summarizes the latest information regarding the molecular organization of the epidermal basement membrane as determined by immunoelectron microscopy as well as the blistering diseases that occur in the epidermal basement membrane zone.
    背景与目标: :表皮基底膜是位于表皮和真皮之间的特殊结构。最近的研究阐明了基底膜的生物学作用及其在大疱性疾病中的病理生理学影响。要了解基底膜的功能,必须有关于表皮基底膜超微结构分子组织的清晰准确信息。免疫电子显微镜技术是一项功能强大的技术,并且是唯一可用于阐明分子的超微结构定位或取向的方法。这篇综述总结了由免疫电子显微镜确定的有关表皮基底膜分子组织的最新信息,以及在表皮基底膜区发生的水疱性疾病。
  • 【角质形成细胞培养中的水泡:与分化相关的正常现象。】 复制标题 收藏 收藏
    DOI: 复制DOI
    作者列表:Chapman SJ,Eady RA
    BACKGROUND & AIMS: :Blisters have previously been observed in keratinocyte cultures depleted of vitamin A, and in cultures of keratinocytes from patients with epidermolysis bullosa. We have found that blistering may occur in keratinocyte cultures from normal human epidermis, grown under standard conditions, and our aim was to further characterize the mechanism of blister formation. Keratinocytes were seeded at 10(5) cells per 35 mm collagen-coated dish with a 3T3 feeder layer. Blisters were macroscopic, fluid-filled structures which formed irrespective of donor site, or donor age, and were noted on various alternative substrates (collagen, 3T3 + plastic, plastic alone). Blistering commenced around day 12, prior to confluency, and new blisters were formed for up to 5 weeks post-plating. Maximal numbers (up to 70 per dish) were present around days 12 to 20. Cleavage occurred at the cell/collagen interface to form a blister roof composed of 6 to 9 cell layers. The lowest layer appeared metabolically active, but, in contrast to peri-blister regions, lacked hemidesmosomes. The central 2 to 3 layers contained membrane-coating granules and keratohyalin granules while the superficial strata resembled rudimentary corneocytes. Cultures supplemented with 10(-5) M vitamin A formed no blisters, which correlated with suppressed differentiation. Ouabain (10(-7) M) caused blister collapse and a reversible inhibition of new blister formation. We conclude that blisters are a consistent finding in keratinocyte cultures grown under standard conditions. Their formation may be associated with active transport and triggered during differentiation. Further examination of this phenomenon might shed light on whether differentiation itself has an influence on keratinocyte attachment to substrate.
    背景与目标: :以前在缺乏维生素A的角质形成细胞培养物中以及在大疱性表皮松解症患者的角质形成细胞培养物中观察到水泡。我们发现在正常条件下生长的正常人表皮的角质形成细胞培养物中可能会出现水泡,我们的目标是进一步表征水泡形成的机制。角质形成细胞以每35毫米带有3T3饲养层的胶原涂层培养皿中的10(5)个细胞接种。水泡是宏观的,充满液体的结构,与供体部位或供体年龄无关而形成,并且在各种替代性底物(胶原蛋白,3T3塑料,仅塑料)上被注意到。在融合前的第12天左右开始起泡,并在接种后长达5周的时间内形成新的水泡。在第12至20天左右出现最大数量(每个培养皿最多70个)。在细胞/胶原蛋白界面发生裂解,形成由6至9个细胞层组成的水泡屋顶。最低层表现出代谢活性,但与水泡周围区域相比,缺乏半桥粒。中央的2至3层包含膜包衣颗粒和角膜透明质酸颗粒,而表层则类似于原始角质细胞。补充有10(-5)M维生素A的培养物未形成水泡,这与抑制分化相关。瓦巴因(10(-7)M)引起水泡塌陷和新水泡形成的可逆抑制。我们得出结论,水泡是在标准条件下生长的角质形成细胞培养物中的一致发现。它们的形成可能与主动转运有关,并在分化过程中被触发。对该现象的进一步检查可能会揭示分化本身是否会影响角质形成细胞对基质的附着。
  • 【完全FcRn依赖性,可用于自身免疫性皮肤水疱病的静脉Ig治疗。】 复制标题 收藏 收藏
    DOI:10.1172/JCI24394 复制DOI
    作者列表:Li N,Zhao M,Hilario-Vargas J,Prisayanh P,Warren S,Diaz LA,Roopenian DC,Liu Z
    BACKGROUND & AIMS: :Numerous mechanisms of action have been proposed for intravenous Ig (IVIG). In this study, we used IgG passive transfer murine models of bullous pemphigoid (BP), pemphigus foliaceus (PF), and pemphigus vulgaris (PV) to test the hypothesis that the effect of IVIG in autoantibody-mediated cutaneous bullous diseases is to accelerate the degradation of pathogenic IgG by saturation of the MHC-like Fc receptor neonatal Fc receptor (FcRn). BP, PF, and PV are organ-specific antibody-mediated diseases in which autoantibodies target the hemidesmosomal antigen BP180 and desmosomal antigens Dsg1 and Dsg3, respectively. Antibodies against BP180, Dsg1, and Dsg3, when injected into neonatal mice, induce the BP, PF, and PV disease phenotypes, respectively. We found that FcRn-deficient mice were resistant to experimental BP, PF, and PV. Circulating levels of pathogenic IgG in FcRn-deficient mice were significantly reduced compared with those in WT mice. Administration of high-dose human IgG (HDIG) to WT mice also drastically reduced circulating pathogenic IgG levels and prevented blistering. In FcRn-deficient mice, no additional protective effect with HDIG was realized. These data demonstrate that the therapeutic efficacy of HDIG treatment in the pemphigus and pemphigoid models is dependent on FcRn. Thus, FcRn is a promising therapeutic target for treating such IgG-mediated autoimmune diseases.
    背景与目标: :已经提出了多种作用机制用于静脉注射Ig(IVIG)。在这项研究中,我们使用了大疱性天疱疮(BP),天疱疮天疱疮(PF)和寻常型天疱疮(PV)的IgG被动转移小鼠模型来检验IVIG在自身抗体介导的皮肤大疱性疾病中的作用是加速免疫球蛋白MHC样Fc受体新生Fc受体(FcRn)饱和引起的致病性IgG降解。 BP,PF和PV是器官特异性抗体介导的疾病,其中自身抗体分别靶向半桥粒抗原BP180和桥粒抗原Dsg1和Dsg3。当将抗BP180,Dsg1和Dsg3的抗体注射入新生小鼠后,它们分别诱导BP,PF和PV疾病表型。我们发现,FcRn缺陷型小鼠对实验性BP,PF和PV具有抗性。与WT小鼠相比,FcRn缺陷型小鼠的致病性IgG循环水平显着降低。向野生型小鼠施用大剂量人IgG(HDIG)还可以大大降低循环中的致病性IgG水平并防止起泡。在FcRn缺陷型小鼠中,没有实现HDIG的其他保护作用。这些数据证明,HDIG在天疱疮和天疱疮模型中的治疗功效取决于FcRn。因此,FcRn是用于治疗这种IgG介导的自身免疫疾病的有希望的治疗靶标。
  • 【与一步式自蚀刻胶粘剂结合在一起的搪瓷中的渗透性起泡。】 复制标题 收藏 收藏
    DOI:10.1177/154405910408300404 复制DOI
    作者列表:Tay FR,Lai CN,Chersoni S,Pashley DH,Mak YF,Suppa P,Prati C,King NM
    BACKGROUND & AIMS: :One-step self-etch adhesives behave as permeable membranes after polymerization, permitting water to move through the cured adhesives. We hypothesize that osmotic blistering occurs in bonded enamel when these adhesives are used without composite coupling. Tooth surfaces from extracted human premolars were bonded with 5 one-step self-etch adhesives. They were immersed in distilled water or 4.8 M CaCl(2), and examined by stereomicroscopy, field-emission/environmental SEM, and TEM. Water blisters were observed in bonded enamel but not in bonded dentin when specimens were immersed in water. They collapsed when water was subsequently replaced with CaCl(2). Blisters were absent from enamel in specimens that were immersed in CaCl(2) only. Water trees were identified from adhesive-enamel interfaces. Osmotic blistering in enamel is probably caused by the low water permeability of enamel. This creates an osmotic gradient between the bonded enamel and the external environment, causing water sorption into the interface.
    背景与目标: :一步式自蚀刻胶粘剂在聚合后表现为可渗透膜,使水能够流过固化的胶粘剂。我们假设,当使用这些粘合剂而没有复合偶联剂时,粘结的瓷釉中会发生渗透性起泡。用5种单步自蚀刻粘合剂将提取的人类前磨牙的牙齿表面粘合在一起。将它们浸入蒸馏水或4.8 M CaCl(2)中,并通过体视显微镜,场发射/环境SEM和TEM进行检查。将标本浸入水中时,在粘合的瓷釉中观察到水泡,但在粘合的牙本质中未观察到水泡。随后将水替换为CaCl(2)时,它们崩溃了。仅浸入CaCl(2)的标本中的搪瓷上没有气泡。从粘合剂-瓷釉界面中鉴定出水树。搪瓷中的渗透性起泡可能是由于搪瓷的低透水性引起的。这会在粘结的瓷釉和外部环境之间产生渗透梯度,从而导致水吸附到界面中。
  • 【自身免疫性水疱病中的新型免疫调节药物。】 复制标题 收藏 收藏
    DOI:10.1016/s0733-8635(05)70305-3 复制DOI
    作者列表:Korman NJ
    BACKGROUND & AIMS: The autoimmune blistering diseases are a fascinating group of diseases characterized by the presence of blisters involving the skin and mucous membranes. Understanding of the diagnosis, pathophysiology, and advances in treatment of these diseases has grown enormously in recent years. In this article, the author discusses the major clinical and immunopathologic findings in bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, linea IgA bullous disease, and pemphigus. The article focuses on the therapeutic management of patients with autoimmune blistering diseases, including the appropriate treatment of patients, with particular emphasis on the use of immunomodulating and immunosuppresive agents.

    背景与目标: 自身免疫性水疱病是一组引人入胜的疾病,其特征在于存在涉及皮肤和粘膜的水泡。近年来,对这些疾病的诊断,病理生理学和治疗进展的了解已大大增加。在本文中,作者讨论了大疱性天疱疮,粘膜膜天疱疮,表皮松解性大疱性水疱,线虫IgA大疱性疾病和天疱疮的主要临床和免疫病理学发现。本文着重于自身免疫性水疱病患者的治疗管理,包括对患者的适当治疗,特别着重于免疫调节剂和免疫抑制剂的使用。

  • 【在自身免疫性水疱性疾病中使用皮质类固醇。】 复制标题 收藏 收藏
    DOI:10.1016/j.iac.2012.04.008 复制DOI
    作者列表:Frew JW,Murrell DF
    BACKGROUND & AIMS: :Corticosteroids, while providing rapid remission and ongoing control of symptoms of autoimmune blistering diseases (AIBD), have numerous potentially serious acute and long-term side effects. Evidence-based medicine has reevaluated the various types of corticosteroids and forms of corticosteroid delivery in AIBD to ascertain whether any advantages of specific delivery systems or regimens exist. Careful monitoring of patients and simple preventive measures are effective in minimizing the adverse outcomes associated with their use. This article outlines the current level of evidence for corticosteroid use in AIBDs, and discusses appropriate investigations and interventions to minimize or prevent the associated adverse effects.
    背景与目标: :皮质类固醇可提供快速缓解并持续控制自身免疫性水疱病(AIBD)症状,但具有许多潜在的严重急性和长期副作用。循证医学已经重新评估了AIBD中各种类型的皮质类固醇和皮质类固醇的递送形式,以确定特定的递送系统或治疗方案是否存在任何优势。仔细监视患者并采取简单的预防措施可有效减少与使用相关的不良后果。本文概述了AIBD中使用皮质类固醇的现有证据水平,并讨论了适当的调查和干预措施,以最大程度地减少或预防相关的不良影响。
  • 【苯妥英钠对减轻大疱性表皮松解的水疱作用4例报道。】 复制标题 收藏 收藏
    DOI: 复制DOI
    作者列表:Sasidharan CK,Vijayakumar M,Vinodkumor MS
    BACKGROUND & AIMS: :To evaluate the effect of phenytoin in reducing of patients with epidermolysis bullosa four newborn babies were studied after thorough clinical evaluation including detailed history and relevant investigations. All of them were put on oral phenytoin sodium, in the usual antiepileptic dose and were followed up. All of the babies had significant reduction in the number of lesions.
    背景与目标: :为了评估苯妥英钠在大疱性表皮松解症患者中的减少作用,经过全面的临床评估,包括详细的病史和相关研究,对4例新生儿进行了研究。所有患者均按常规抗癫痫药剂量口服苯妥英钠,并进行随访。所有婴儿的病灶数量均明显减少。
  • 【针对起泡的化学战剂的医学防御。】 复制标题 收藏 收藏
    DOI: 复制DOI
    作者列表:Smith WJ,Dunn MA
    BACKGROUND & AIMS: :First used in World War I, chemical blistering agents present a serious medical threat that has stimulated renewed interest in the light of extensive use in recent conflicts. Current medical management cannot yet prevent or minimize injury from the principal agent of concern--sulfur mustard. Research directed at this goal depends on defining effective intervention in the metabolic alterations induced by exposure to sulfur mustard.
    背景与目标: :起泡剂首次用于第一次世界大战,由于最近在冲突中被广泛使用,化学起泡剂带来了严重的医学威胁,激发了人们的新兴趣。当前的医学管理尚不能防止或减少主要关注因素-硫芥末的伤害。针对该目标的研究取决于对暴露于硫芥子气引起的代谢变化进行有效干预。
  • 【转移性卵巢癌相关的表皮下水疱性疾病伴有针对p200真皮抗原和层粘连蛋白5的γ2亚基的自身抗体,显示出异常的临床特征。】 复制标题 收藏 收藏
    DOI:10.1111/j.1365-2133.2008.08483.x 复制DOI
    作者列表:Mitsuya J,Hara H,Ito K,Ishii N,Hashimoto T,Terui T
    BACKGROUND & AIMS: :Anti-p200 pemphigoid is an autoimmune subepidermal blistering disease characterized by autoantibodies to an unknown 200-kDa acidic noncollagenous glycoprotein of the lower lamina lucida, whereas antilaminin 5 mucous membrane pemphigoid is characterized by autoantibodies to a major basement membrane extracellular matrix, laminin 5. We report a 64-year-old Japanese woman with a subepidermal blistering disease associated with lymph node metastasis of ovarian clear cell carcinoma 10 years after its surgical treatment. Clinical features showed severe blisters and erosions on multiple mucous membranes (i.e. lip, oral cavity, nose, eye, genitalia and anus) and on both the periungual and subungual regions. This is the first report in which an immunoblot analysis revealed the unusual combination of autoantibodies to both the p200 antigen and the gamma 2 subunit of laminin 5.
    背景与目标: Anti-p200天疱疮是一种自身免疫性表皮下水疱性疾病,其特征是针对下部lucina lucida的未知200 kDa酸性非胶原糖蛋白的自身抗体,而antilaminin 5粘膜天疱疮的特征是针对主要基底膜细胞外基质laminin 5的自身抗体。我们报道了一名64岁的日本女性,患有10年后的卵巢透明细胞癌淋巴结转移相关的表皮下水疱性疾病。临床表现为在多个粘膜(即嘴唇,口腔,鼻子,眼睛,生殖器和肛门)以及在唇周和舌下区域均出现严重的水泡和侵蚀。这是第一份免疫印迹分析显示针对p200抗原和层粘连蛋白5的gamma 2亚基的自身抗体异常组合的报告。
  • 【在表皮祖细胞中转录因子Ovol2的过表达导致皮肤起泡。】 复制标题 收藏 收藏
    DOI:10.1016/j.jid.2017.02.985 复制DOI
    作者列表:Lee B,Watanabe K,Haensel D,Sui JY,Dai X
    BACKGROUND & AIMS: -2
    背景与目标: -2
  • 【一例表皮下水疱病,对多个层粘连蛋白亚单位有自身抗体,后来又发展了与膜性肾小球肾病相关的IV型胶原α-5链的自身抗体。】 复制标题 收藏 收藏
    DOI:10.2340/00015555-2059 复制DOI
    作者列表:Sueki H,Sato Y,Ohtoshi S,Nakada T,Yoshimura A,Tateishi C,Borza DB,Fader W,Ghohestani RF,Hirako Y,Koga H,Ishii N,Tsuchisaka A,Qian H,Li X,Hashimoto T
    BACKGROUND & AIMS: :We report a 68-year-old Japanese female patient with subepidermal blistering disease with autoantibodies to multiple laminins, who subsequently developed membranous glomerulonephropathy. At skin disease stage, immunofluorescence demonstrated IgG anti-basement membrane zone antibodies reactive with dermal side of NaCl-split skin. Immunoblotting of human dermal extract, purified laminin-332, hemidesmosome-rich fraction and laminin-521 trimer recombinant protein (RP) detected laminin γ-1 and α-3 and γ-2 subunits of laminin-332. Three years after skin lesions disappeared, nephrotic symptoms developed. Antibodies to α-3 chain of type IV collagen (COL4A3) were negative, thus excluding the diagnosis of Goodpasture syndrome. All anti-laminin antibodies disappeared. Additional IB and ELISA studies of RPs of various COL4 chains revealed reactivity with COL4A5, but not with COL4A6 or COL4A3. Although diagnosis of anti-laminin γ-1 (p200) pemphigoid or anti-laminin-332-type mucous membrane pemphigoid could not be made, this case was similar to previous cases with autoantibodies to COL4A5 and/or COL4A6.
    背景与目标: :我们报道了一名68岁的日本女性,患有表皮下水疱病,对多种层粘连蛋白具有自身抗体,随后发展成膜性肾小球肾病。在皮肤疾病阶段,免疫荧光显示IgG抗基底膜区抗体可与NaCl分裂的皮肤的真皮侧发生反应。人类皮肤提取物,纯化的层粘连蛋白332,富含半脂质体的级分和层粘连蛋白521三聚体重组蛋白(RP)的免疫印迹检测到层粘连蛋白332的层粘连蛋白γ-1和α-3和γ-2亚基。皮肤病变消失三年后,出现肾病症状。 IV型胶原的α-3链抗体(COL4A3)呈阴性,因此排除了Goodpasture综合征的诊断。所有抗层粘连蛋白抗体均消失。对各种COL4链的RP进行的其他IB和ELISA研究表明,它与COL4A5有反应性,但与COL4A6或COL4A3没有反应性。尽管无法诊断出抗lamininγ-1(p200)天疱疮或抗laminin-332型粘膜天疱疮,但该病例与先前具有COL4A5和/或COL4A6自身抗体的病例相似。
  • 【颗粒酶B抑制可降低自身免疫性水疱性疾病的疾病严重程度。】 复制标题 收藏 收藏
    DOI:10.1038/s41467-020-20604-3 复制DOI
    作者列表:Hiroyasu S,Zeglinski MR,Zhao H,Pawluk MA,Turner CT,Kasprick A,Tateishi C,Nishie W,Burleigh A,Lennox PA,Van Laeken N,Carr NJ,Petersen F,Crawford RI,Shimizu H,Tsuruta D,Ludwig RJ,Granville DJ
    BACKGROUND & AIMS: :Pemphigoid diseases refer to a group of severe autoimmune skin blistering diseases characterized by subepidermal blistering and loss of dermal-epidermal adhesion induced by autoantibody and immune cell infiltrate at the dermal-epidermal junction and upper dermis. Here, we explore the role of the immune cell-secreted serine protease, granzyme B, in pemphigoid disease pathogenesis using three independent murine models. In all models, granzyme B knockout or topical pharmacological inhibition significantly reduces total blistering area compared to controls. In vivo and in vitro studies show that granzyme B contributes to blistering by degrading key anchoring proteins in the dermal-epidermal junction that are necessary for dermal-epidermal adhesion. Further, granzyme B mediates IL-8/macrophage inflammatory protein-2 secretion, lesional neutrophil infiltration, and lesional neutrophil elastase activity. Clinically, granzyme B is elevated and abundant in human pemphigoid disease blister fluids and lesional skin. Collectively, granzyme B is a potential therapeutic target in pemphigoid diseases.
    背景与目标: :类天疱疮疾病是指一组严重的自身免疫性皮肤起泡疾病,其特征在于表皮下起泡和自身抗体诱导的表皮粘附性丧失,免疫细胞在真皮-表皮交界处和上层真皮中浸润。在这里,我们使用三种独立的小鼠模型探讨了分泌免疫细胞的丝氨酸蛋白酶粒酶B在类天疱疮疾病发病机理中的作用。与对照相比,在所有模型中,颗粒酶B的敲除或局部药理抑制作用均显着降低了总的起泡面积。体内和体外研究表明,颗粒酶B通过降解真皮-表皮连接中关键的锚定蛋白(对真皮-表皮的粘附是必需的)而有助于起泡。此外,粒酶B介导IL-8 /巨噬细胞炎性蛋白2分泌,病变中性粒细胞浸润和病变中性粒细胞弹性蛋白酶活性。在临床上,人类天疱疮疾病的水疱液和病灶皮肤中的颗粒酶B升高并且含量很高。总的来说,粒酶B是类天疱疮疾病的潜在治疗靶标。
  • 【遗传性皮肤起泡中角蛋白5的保守螺旋终止肽中的突变。】 复制标题 收藏 收藏
    DOI:10.1038/356244a0 复制DOI
    作者列表:Lane EB,Rugg EL,Navsaria H,Leigh IM,Heagerty AH,Ishida-Yamamoto A,Eady RA
    BACKGROUND & AIMS: :In the hereditary blistering condition epidermolysis bullosa simplex, the skin blisters on trauma following rupture of epidermal basal cells. Clinical variations range from severely incapacitating, especially in early childhood, to mild forms that may not even present clinically. Dowling-Meara epidermolysis bullosa simplex is characterized by clusters of epidermal blisters and keratin clumping in the cytoplasm; recent reports describe potentially causal mutations in keratin 14 (refs 2, 3). Here we describe a 'complementary' mutation at the other end of the other keratin expressed by these cells (K5, coexpressed with K14), a change from a Glu to a Gly in the helix termination peptide, detected by altered antibody binding and confirmed by sequencing using the polymerase chain reaction. The two conserved helix boundary peptides are predicted to be essential for filament assembly, and the requirement for two complementary (type I and type II) keratins is absolute. Epidermolysis bullosa simplex diseases demonstrate the function of the keratin cytoskeleton in resisting compaction stresses which otherwise lead to cell lysis.
    背景与目标: :在大疱性表皮松解的遗传性起泡情况下,表皮基底细胞破裂后,皮肤因创伤而起水泡。临床变化范围从严重丧失工作能力(尤其是在儿童早期丧失工作能力)到可能甚至在临床上都不存在的轻度形式。 Dowling-Meara表皮松解性大疱性单纯疱疹的特征是在细胞质中有表皮水泡簇和角蛋白聚集。最近的报道描述了角蛋白14中潜在的因果突变(参考文献2、3)。在这里,我们描述了由这些细胞表达的其他角蛋白(K5,与K14共表达)另一端的“互补”突变,螺旋终止肽从Glu变为Gly的变化,通过改变抗体结合来检测并通过使用聚合酶链反应进行测序。预测这两个保守的螺旋边界肽对于长丝组装是必不可少的,并且对两个互补的(I型和II型)角蛋白的需求是绝对的。表皮松解性大疱性单纯性疾病证明了角蛋白细胞骨架在抵抗压紧应力方面的功能,否则该压紧应力会导致细胞溶解。
  • 【从茄子茄中分离出的番茄起泡花叶病毒高度分离株。】 复制标题 收藏 收藏
    DOI:10.1007/s11262-016-1288-z 复制DOI
    作者列表:Blawid R,Hayashi EA,Rezende JA,Kitajima EW,Nagata T
    BACKGROUND & AIMS: :The complete genome of a tymovirus infecting Solanum violaefolium was sequenced. The genome comprised 6284 nt, with a 5'-UTR of 137 nt and a comparatively longer 3'-UTR of 121 nt. Sequence analysis confirmed three ORFs encoding a movement protein, a polyprotein, and a coat protein (CP). The isolate was considered to be the Tomato blistering mosaic virus (ToBMV) based on a CP amino acid sequence identity of 95.3 %. The nucleotide sequence of the complete genome of the S. violaefolium isolate, however, differed markedly from the other two reported ToBMV isolates, with identities of 76.6 and 76.3 %, below one of the demarcation criteria of the genus Tymovirus (overall genome identity of 80 %). No recombination signals were detected in the genome of this isolate. The high identity of the CP amino acid sequence and similar host responses suggest that the S. violaefolium isolate belongs to the same species as the Tomato blistering mosaic virus. The sequence analysis of this ToBMV isolate thus suggests that the demarcation criterion of 80 % overall genome sequence identity in the genus Tymovirus may require revision.
    背景与目标: :对感染小菜蛾的鼓膜病毒的完整基因组进行了测序。基因组包含6284nt,具有5'-UTR为137nt和相对更长的3'-UTR为121nt。序列分析证实了三个ORF,它们编码运动蛋白,多蛋白和外壳蛋白(CP)。基于95.3%的CP氨基酸序列同一性,该分离物被认为是番茄起泡花叶病毒(ToBMV)。但是,紫罗兰链球菌分离物完整基因组的核苷酸序列与其他两个报道的ToBMV分离物显着不同,同一性分别为76.6和76.3%,低于胸膜炎病毒属的一种划界标准(总体基因组同一性为80)。 %)。在该分离物的基因组中未检测到重组信号。 CP氨基酸序列的高度同一性和相似的宿主反应表明,紫堇链球菌分离株与番茄起泡花叶病毒属于同一物种。因此,对该ToBMV分离株的序列分析表明,在Tymovirus属中,总基因组序列同一性为80%的分界标准可能需要修改。

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