Hereditary neuropathy with liability to pressure palsies

Summary Clinical, neurophysiological and pathological investigations were carried out in 11 affected members of 2 families with hereditary neuropathy with liability to pressure palsies (HNPP). The observations were related to findings in 261 cases of 47 families published in the literature. It was concluded that HNPP is a nosological entity characterized by the following diagnostic criteria: (1) an autosomal dominant inheritance; (2) the clinical presentation of a recurrent mononeuropathy simplex or multiplex, frequently related to an inadequate trauma to peripheral nerves; (3) a significant slowing of motor and sensory conduction velocity in clinically affected, but also in clinically unaffected nerves; (4) characteristic morphological findings in sural nerve biopsy featuring tomaculous swellings of myelin sheaths, transnodal myelination and segmental demyelination. The pathogenesis of HNPP is not clear. Hypothetical explanations of the pathogenesis of HNPP are discussed.In memory of Albert Bischoff (1921–1981), Professor of Neurology, University of Berne     

p53、bcl-2蛋白和p-gp在鼻咽鳞癌中表达的研究

 目的　研究 p5 3、bcl 2蛋白和 p gp在鼻咽鳞癌组织中的表达及其临床病理特征的关系 ,探讨其在预后评估中的意义。方法　采用免疫组化SP法检测 6 4例鼻咽鳞癌组织中p5 3、bcl 2和 p gp的表达。结果　鼻咽鳞癌组织中 p5 3、bcl 2和p gp阳性率分别为76 .5 6 % (4 9/ 6 4 )、89.0 6 % (5 7/ 6 4 )、17.19% (11/6 4 )。p5 3、bcl 2蛋白过表达均与颈部淋巴结转移、组织分化程度有关 (P <0 .0 1)。p gp过表达与bcl 2蛋白过表达呈正相关 (P <0 .0 5 ) ,p5 3蛋白过表达与bcl 2蛋白过表达呈正相关 (P <0 .0 0 1)。结论　p5 3和bcl 2蛋白均参与鼻咽鳞癌的发生与分化 ,其表达水平的不同有助于预后判断 ;未经治疗的鼻咽鳞癌存在原发性耐药     

贲门癌组织中P-糖蛋白和p53蛋白的表达及其意义

目的　探讨贲门癌组织中P -糖蛋白 (p -gp)和p5 3蛋白共同表达的临床意义 ,研究其与贲门癌生物学行为的关系。方法　采用免疫组化方法 (二步法 )检测 66例术前未经化疗的贲门癌组织中多药耐药基因产物P -糖蛋白和 p5 3蛋白的表达情况。结果　P -gp和 p5 3在贲门癌组织中表达率分别为 3 6.4% (2 4/66)和 5 1.5 % (3 4/66)。P -gp表达与贲门癌组织学类型、临床分期和淋巴结转移均无关 (P >0 .0 5 ) ;p5 3除与淋巴结转移有关外 (P <0 .0 5 ) ,与其组织学类型和临床分期均无关 (P >0 .0 5 )。p5 3表达阳性病例P -gp表达率明显高于 p5 3表达阴性病例 ,即 83 .3 %P -gp阳性表达患者 p5 3表达阳性。 结论　P -gp和 p5 3常共同表达于贲门癌组织中 ,可作为判断贲门癌患者预后和临床耐药的 1个可靠指标     

结核分枝杆菌热休克蛋白65与汉坦病毒G2糖蛋白融合基因真核表达载体的构建与表达

目的 构建以结核分枝杆菌热休克蛋白65（HSP65）和汉坦病毒G2糖蛋白重组融合基因为基础的真核表达载体。为进一步研究其在结核病和出血热防治中的作用奠定基础。方法 采用聚合酶链反应从结核分枝杆菌H37Rv株基因中．扩增出HSP65的编码基因，从T-H8205G2质粒扩增出G2糖蛋白的编码基因．经相应限制性核酸内切酶消化后．插入真核表达载体pcDNA3．1／His，并将此重组质粒通过脂质体介导转染NIH3T3细胞．用SDS-PAGE、免疫组织化学及免疫荧光方法分别测定表达产物的相对分子量及特异性。结果 获得正向插入的pcDNA3.1/ His-HSP65-G2重组表达质粒，表达产物的相对分子量为105kD．与理论预期大小一致．并且可与汉坦病毒H8205株的腹水抗体和HSP65单抗起特异反应。表明构建的pcDNA3．1／His-HSP65-G2能在哺乳动物细胞中表达并具有抗原性。结论 编码HSP65和G2抗原基因的重组基因的真核表达载体构建成功。     

松口蘑发酵菌丝体糖蛋白MTS03对人乳腺癌细胞MCF-7的体外增殖抑制作用研究

 目的研究松口蘑发酵菌丝体糖蛋白MTS03的理化性质及抗肿瘤作用。方法采用SDS-PAGE鉴定MTS03的纯度及相对分子质量,氨基酸自动分析仪分析其中的氨基酸组成;MTT法研究糖蛋白MTS03体外对人乳腺癌细胞MCF-7的增殖抑制率,同时考察MTS03对人正常肝细胞L-02的杀伤率,评价其安全性。结果SDS-PAGE电泳鉴定为单一组分,测定其相对分子质量约为2.37×10⁴,经氨基酸自动分析仪分析含有17种氨基酸;MTS03对MCF-7具有直接的细胞毒作用,在一定的浓度范围内呈现量效和时效关系,MTS03对MCF-7作用24,48,72h的IC₅₀分别为25.81,12.46,8.86mg·L^-1,而对正常肝细胞的IC₅₀分别为1366.49,190.60,95.58mg·L^-1,对MCF-7的治疗指数TI分别为54.16,18.74,12.25。结论MTS03对于体外培养的乳腺癌细胞具有良好的直接杀伤作用,而对正常肝细胞的毒性较小,可能是一种高效低毒的生物活性物质。     

HB-Ⅰa冻干脂质体粒径及其分布的研究

研究了降温速率、保护剂种类与浓度、复溶溶液对HB-Ⅰa冻干脂质体粒径的影响.由逆相蒸发法制得的HB-Ⅰa脂质体大多为大单室和多室脂质体,粒径主要分布在1～10μm之间.约20K/min的降温速率对HB-Ⅰa脂质体冻干较为有利;蔗糖的保护效果较优,甘露醇次之,PVP和甘氨酸相对较差,蔗糖和甘氨酸组成的二元保护剂保护效果较蔗糖更好;缓冲溶液较纯水或蔗糖溶液的复溶效果好.     

Inflammation and arthritis: perspectives of the glycobiologist

This review focuses on the role of glycosylation during the development of joint inflammation and degeneration. Although glycoproteins and glycan-binding proteins have essential functions in bone and cartilage, and in the inflammatory process, their exact roles are still uncertain due to the vast complexity of carbohydrate structures. Glycosylated epitopes have been shown to play a role in the induction of arthritis in animal models. Currently available drugs are aimed at the protection of cartilage and bone structures but new developments in this area should take into account the tight and specific interactions between bone and cartilage. It is anticipated that new agents will help to remodel damaged joints, based on knowledge of cartilage and bone turnover and on the exact role of glycosylated molecules and cell surface receptor glycoproteins in these processes. Highly sensitive imaging techniques and well-characterized In vivo models will accelerate this development.     

Identification and localisation of glycosyl moieties of surface glycoproteins of teliospore wall of Karnal bunt (Tilletia indica) of wheat

This article aims to characterise and localise the glycosyl moieties of teliospore wall of Tilletia indica a quarantined fungal pathogens by biochemical and immunological approaches. Chemical enzyme modifier studies, followed by determination of structural configuration using phase contrast and SEM after periodate treatment, showed antigenic entities are glycoprotein in nature. Further characterisation using sodium dodecyl sulphate-polyacrylamide gel electrophroesis (SDS-PAGE) glycoprotein staining and western blotting using anti-teliospore antibodies showed two common proteins of molecular weight 28 and 40 kDa, which is also suggestive of glycoprotein nature of antigenic entities of teliospore wall. To study the binding patterns and localisation of glycosyl moieties on the teliospore walls, fluorescein isothiocyanate (FITC) labelled lectins [Wheat Germ Agglutinin (WGA) and Concanavilin A (Con A)] and anti-teliospore antibodies were used. The patterns of WGA and anti-teliospore antibodies binding with teliospore wall are almost similar and hence it is quite reasonable to suggest that immunodominant glycosyl entities of teliospore wall are acetylglucosamine in nature.     

Developmental defects in zebrafish for classification of EGF pathway inhibitors

One of the major challenges when testing drug candidates targeted at a specific pathway in whole animals is the discrimination between specific effects and unwanted, off-target effects. Here we used the zebrafish to define several developmental defects caused by impairment of Egf signaling, a major pathway of interest in tumor biology. We inactivated Egf signaling by genetically blocking Egf expression or using specific inhibitors of the Egf receptor function. We show that the combined occurrence of defects in cartilage formation, disturbance of blood flow in the trunk and a decrease of myelin basic protein expression represent good indicators for impairment of Egf signaling. Finally, we present a classification of known tyrosine kinase inhibitors according to their specificity for the Egf pathway.