结合非胶原性黏合蛋白的病原菌黏附素的研究进展

病原菌感染宿主细胞一般都依赖细菌表面的特定分子(黏附素)与胞外基质的相互作用来实现最初的吸附过程,其中细菌结合非胶原性黏附蛋白的黏附素分子主要包括两类:与纤连蛋白结合的细菌纤连蛋白结合蛋白以及与层黏连蛋门结合的层黏连蛋白结合蛋白.此文重点介绍了这两类黏附素的分子特点以及在感染过程中所起的作用.     

肝脏硬度、层粘连蛋白及AFP值对HBV相关HCC的诊断价值

目的 评估血清甲胎蛋白（alpha-fetoprotein,AFP）、肝脏弹性（也称为硬度）测量值（liver stiffness measurement,LSM）、层粘连蛋白（laminin,LN）检测在HBV相关性肝细胞性肝癌（hepatocellular carcinoma,HCC）诊断中价值。方法 采用回顾性研究方法,纳入2013年10月至2021年3月在延安大学附属医院及延安市第二人民医院就诊的HBV感染者,行上腹部增强MRI或增强CT等检查诊断为肝硬化和肝癌的患者。检查肝纤维化四项LSM及AFP值等指标。采用统计学方法分析两组各指标的差异,Logistic单因素及多因素分析筛选诊断HCC有价值的指标。结果 两组透明质酸（hualuronic acid,HA）、Ⅳ型胶原蛋白（collagen Ⅳ,CⅣ）、Ⅲ型前胶原（procollagen type Ⅲ,PCⅢ）、LN、AFP及LSM水平差异,均有统计学意义（P均<0.05）。多因素分析显示,AFP、LN、LSM是HCC的独立危险因素（P均<0.05）。并经ROC曲线分析发现均有诊断价值,AFP的ROC曲线下面积为...     

糖尿病肾病患者血清Ⅳ型胶原和层粘连蛋白水平的变化及临床价值

目的探讨糖尿病肾病(DN)患者血清中Ⅳ型胶原(C-Ⅳ)和层粘连蛋白(LN)水平的变化及其临床价值。方法用放射免疫法测定81例糖尿病(DM)患者(男48例,女33例)的血清C-Ⅳ和LN含量,分为正常白蛋白尿组36例,微量白蛋白尿组29例和大量白蛋白尿组16例,并以50例健康体检者为对照。用直线相关分析糖尿病患者血清C-Ⅳ、LN与其它临床指标之间的相关性。结果81例DM患者血清C-Ⅳ[(74.6±24.2μg/L]、LN[(137.1±29.5)μg/L]水平明显高于对照组[(45.4±7.9)、(98.6±15.4)μg/L,P<0.01];DM各亚组患者血清C-Ⅳ、LN水平均明显高于对照组(P<0.05,P<0.01);DM各组间血清C-Ⅳ、LN水平随尿白蛋白量的增加而升高,差异有统计学意义(P<0.05,P<0.01)。直线相关分折提示血清LN及C-Ⅳ水平均与BUN、Cr、HbA1C、UAER相关(P<0.05,P<0.01),血清C-Ⅳ、LN水平与FBG无相关性(P>0.05)。结论血清C-Ⅳ、LN水平与糖尿病肾病病程的发生发展密切相关,联合检测血清C-Ⅳ、LN、HbA1c、UAER可能更早、更全面地反映DN的病变程度。     

67‐kDa Laminin receptor contributes to hypoxia‐induced migration and invasion of trophoblast‐like cells by mediating matrix metalloproteinase‐9

Insufficient trophoblast invasion often occurs in patients experiencing preeclampsia. The 67‐kDa laminin receptor (LR1) is a multifunctional protein that binds to laminin and interacts with the extracellular matrix. We recently demonstrated that LR1 is implicated in trophoblast migration and invasion. However, whether LR1 is involved in hypoxia‐mediated trophoblastic invasion remains unclear and requires further investigation. This study demonstrates that two trophoblast‐like cell lines (JEG3 and BeWo cells) cultured at 3% oxygen exerted enhanced migratory and invasive capabilities as compared with their counterparts exposed to 20% oxygen. LR1 expression was increased in hypoxic JEG3 cells but decreased after transfection with hypoxia‐inducible factor 1 alpha (HIF‐1α) specific siRNA. Moreover, shRNA targeting LR1 mRNA significantly inhibited hypoxia‐induced increase in matrix metalloproteinase (MMP)‐9 activity in JEG3 cells. Forced overexpression of LR1 augmented JEG3 cell migration and invasion, and enhanced MMP‐9 expression and activity. Additionally, the blockade of the MMP‐9 effect with its neutralizing antibody reduced LR1 elevation‐promoted trophoblastic invasion. In summary, this study demonstrates that LR1 contributes to hypoxia‐induced migration and invasion of trophoblast cells at least partly by mediating MMP‐9 in vitro.     

Epidermal Basement Membrane: Its Molecular Organization and Blistering Disorders

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.     

Limb-girdle muscular dystrophy type 2I is not rare in Taiwan

Alpha-dystroglycanopathy is caused by the glycosylation defects of α-dystroglycan (α-DG). The clinical spectrum ranges from severe congenital muscular dystrophy (CMD) to later-onset limb girdle muscular dystrophy (LGMD). Among all α-dystroglycanopathies, LGMD type 2I caused by FKRP mutations is most commonly seen in Europe but appears to be rare in Asia. We screened uncategorized 40 LGMD and 10 CMD patients by immunohistochemistry for α-DG and found 7 with reduced α-DG immunostaining. Immunoblotting with laminin overlay assay confirmed the impaired glycosylation of α-DG. Among them, five LGMD patients harbored FKRP mutations leading to the diagnosis of LGMD2I. One common mutation, c.948delC, was identified and cardiomyopathy was found to be very common in our cohort. Muscle images showed severe involvement of gluteal muscles and posterior compartment at both thigh and calf levels, which is helpful for the differential diagnosis. Due to the higher frequency of LGMD2I with cardiomyopathy in our series, the early introduction of mutation analysis of FKRP in undiagnosed Taiwanese LGMD patients is highly recommended.     

肺癌层黏连蛋白及其受体表达的临床研究

目的 :探讨肺癌组织层黏连蛋白 (LN)及其受体 (LN -R)的表达和肿瘤转移及预后关系。方法 :采用免疫组织化学技术在 4 2例肺癌患者中研究LN和LN -R的表达。结果 :Ⅰ期患者LN(5 8.30 % )表达高于Ⅱ期 (14 .30 % )及Ⅲ～Ⅳ期 (37.5 2 % )患者 ;无淋巴结肺癌转移患者LN表达率 (6 6 .6 6 % )明显高于有淋巴结转移患者 (2 4 .2 0 % )。有淋巴结肺癌转移患者LN -R表达率 (84 .80 % )明显高于无淋巴结转移患者 (2 2 .2 2 % ) ,这些差异均有统计学意义 (P <0 .0 1)。结论 :LN及其受体 (LN -R)表达在肺癌组织的恶性生物学行为相关 ,可能是临床治疗和预后估计的有用指标。     

Atypical phenotype in two patients with LAMA2 mutations

Congenital muscular dystrophy type 1A is caused by mutations in the LAMA2 gene, which encodes the α2-chain of laminin. We report two patients with partial laminin-α2 deficiency and atypical phenotypes, one with almost exclusive central nervous system involvement (cognitive impairment and refractory epilepsy) and the second with marked cardiac dysfunction, rigid spine syndrome and limb-girdle weakness. Patients underwent clinical, histopathological, imaging and genetic studies. Both cases have two heterozygous LAMA2 variants sharing a potentially pathogenic missense mutation c.2461A>C (p.Thr821Pro) located in exon 18. Brain MRI was instrumental for the diagnosis, since muscular examination and motor achievements were normal in the first patient and there was a severe cardiac involvement in the second. The clinical phenotype of the patients is markedly different which could in part be explained by the different combination of mutations types (two missense versus a missense and a truncating mutation).     

Effects of elevated magnesium and substrate on neuronal numbers and neurite outgrowth of neural stem/progenitor cells in vitro

Because a potential treatment for brain injuries could be elevating magnesium ions (Mg²⁺) intracerebrally, we characterized the effects of elevating external Mg²⁺ in cultures of neonatal murine brain-derived neural stem/progenitor cells (NSCs). Using a crystal violet assay, which avoids interference of Mg²⁺ in the assay, it was determined that substrate influenced Mg²⁺ effects on cell numbers. On uncoated plastic, elevating Mg²⁺ levels to between 2.5 and 10 mM above basal increased NSC numbers, and at higher concentrations numbers decreased to control or lower levels. Similar biphasic curves were observed with different plating densities, treatment durations and length of time in culture. When cells were plated on laminin-coated plastic, NSC numbers were higher even in basal medium and no further effects were observed with Mg²⁺. NSC differentiation into neurons was not altered by either substrate or Mg²⁺ supplementation. Some parameters of neurite outgrowth were increased by elevated Mg²⁺ when NSCs differentiated into neurons on uncoated plastic. Differentiation on laminin resulted in increased neurites even in basal medium and no further effects were seen when Mg²⁺ was elevated. This system can now be used to study the multiple mechanisms by which Mg²⁺ influences neuronal biology.     

HIF-1a,VEGF和LN-R在胃肠间质瘤中的表达及其意义

目的 探讨缺氧诱导因子(HIF-1α)、血管内皮生长因子(vEGF)和层粘连蛋白因子受体(LNR)在胃肠间质瘤(GISTs)中的表达及其在预后判断中的价值.方法 应用免疫组织化学S-P法和Envision二步法方法对95例GISTs检测,观察HIF-1a,VEGF和LN-R.在GISTs中的表达情况.结果 按Flecther标准将95例GISTs分为极低风险(2例),低风险(12例),中度风险(53例)和高度风险(28例),HIF-1a,VEGF的表达率在极低和低风险,中度风险和高度风险GISTT中的表达逐渐升高,3组比较有较显著差异(P＜0.05);而LN-R仅7例表达,在侵透浆膜的中度恶性和高度恶性中占6(83.7%)例表达.肿瘤按直径＜5cm,5～10 cm,＞10cm和核分裂＜5/50HPF,5～10/50HPF,＞10/50HPF分为三组,HIF-1a,VEGF的表达也是渐升高的趋势,且3组比较有较显著差异(P＜0.05).结论 HIF-1a,VEGF和LN-R在GISTs中的表达可能提示肿瘤的恶性程度及其预后,并可作为判断预后的有用标志物.同时可能给GISTs的抗血管分子治疗提供科学依据.