Generalized,severe epidermolysis bullosa simplex caused by a Keratin 5 p.E477K mutation

Epidermolysis bullosa simplex (EBS) is a skin fragility disorder resulting from mutations of structural proteins in the epidermis. We provide a brief report of long‐term survival and reproduction in a mother with EBS due to keratin 5 (KRT5) c.1429G > A (p.E477K) mutation, which causes a particularly severe form of the disease.     

人发角蛋白桥接周围神经缺损的形态学观察

目的 :了解人发角蛋白在神经再生中的作用 ,为临床桥接周围神经缺损寻求新的替代材料。方法 :将 18只新西兰兔的双侧坐骨神经切断 ,造成 10mm缺损 ,一侧用人发角蛋白桥接 (实验组 ) ,另一侧用空硅胶管桥接 (对照组 ) ,术后 1、 2、 3个月通过肉眼观察 ,光镜、电镜和有髓神经密度测定 ,观察、分析神经再生情况。结果 :实验组再生神经均通过 10mm缺损 ,对照组有 2例无神经生长 ;实验组再生神经排列较紧密、有序 ,髓鞘形成早于对照组 ;神经纤维密度明显高于对照组 (P <0 0 1)。结论 :人发角蛋白可促进周围神经再生 ,是桥接周围神经缺损的理想材料。     

Keratin 13基因在喉癌发生中的作用

探讨keratin 13基因在喉癌发生中的作用。方法在keratin 13基因内部及附近选择5个微卫星引物进行LOH分析，于DNA水平间接检测100例喉癌患者中该基因的缺失情况。结果5个STR位点均存在LOH，其中D17S1964E、D17S2092、D17S791、D17S1665及D17S808位点的LOH频率分别为30．48％、26．02％、21．62％、37．66%和21．51％，以D17S1665位点的LOH频率最高，杂合性丢失与临床分期无显著相关。结论Keratin13基因在喉癌的发生中具有重要作用，具体机制有待进一步研究。     

人发角蛋白对脊髓损伤大鼠少突胶质细胞增殖分化效应的影响

目的探讨体内可降解生物材料人发角蛋白(HHK)植入急性冲击性损伤脊髓后,对神经再生过程中少突胶质细胞增殖分化效应的影响。方法采用改制Ⅱ型NYU装置,在建立大鼠急性冲击性脊髓损伤模型基础上,将经过特殊处理后能在体内降解的HHK植入大鼠脊髓损伤部位,对植入后1、4、12、26周损伤脊髓组织进行光学和电镜结构观察。结果第1周时,损伤部位可见少突胶质细胞散在分布于大量浸润的炎症细胞中;第4周时,通过Mallory's磷乌酸苏木素染色,显示HHK周边呈层包绕的增生少突胶质细胞;第12和26周主要为神经再生和髓鞘重建过程,重建中的少突胶质细胞髓鞘内出现较大的空腔,髓鞘层状分离,并形成大小不一的髓鞘小体,重建髓鞘周边可见新生的少突胶质细胞。结论在急性冲击性脊髓损伤后神经再生过程中,HHK对少突胶质细胞的增殖分化及髓鞘再生修复有积极作用,为进一步综合研究HHK对脊髓损伤修复的作用提供了实验依据。     

低温冷冻对皮肤α-tubulin、β-tubulin和cytoskeratin表达的影响

目的:探讨低温冷冻对皮肤骨架蛋白中α-微管蛋白(α-tubu lin)、β-微管蛋白(β-tubu lin)和高分子量角蛋白(cytoskeratin)表达的影响,从而进一步揭示皮肤低温损伤的机制。方法:用-αtubu lin、β-tubu lin、cytoskeratin抗体对5种储存条件下的皮肤作免疫组化染色,采用计算机图像分析技术进行定量分析。并进一步采用透射电镜观察-αtubu lin、β-tubu lin、cytoskeratin的超微结构的变化。结果:α-tubu lin、β-tubu lin、cytoskeratin在4℃组、-20℃组、-80℃组均表达显著下降,且超微形态结构及分布改变明显。在-196℃组储存时三种成分含量下降较少,形态改变亦不显著。结论:皮肤的低温损伤与骨架蛋白-αtubu lin、β-tubu lin、cytoskeratin的破坏有关。     

Phenotypic Cytoskeletal Heterogeneity in Melanoma, a Challenge to the Surgical Pathologist Evaluating a Poorly Differentiated Neoplasm: An Illustrative Case

Characterization of poorly differentiated neoplasms can be a challenging task for the surgical pathologist. It is essential that the entire spectrum of immunomorphologic findings of various tumors be recognized to avoid improper characterization of a given neoplasm, which may in turn adversely affect patient management. Tumor characterization is complicated by the immunomorphologic transformations that malignant cells may undergo by virtue of which they may depart from expression of expected features and acquire new, unexpected characteristics. Traditionally, amelanotic melanomas have been difficult to characterize because of the diversity of their light microscopic morphology (epithelioid, spindle, and combined varieties). As a result, several other neoplasms are usually considered in the differential diagnosis. This report describes a primarily spindle-cell amelanotic melanoma that created a diagnostic dilemma, which could only be resolved by combining the information obtained from extensive evaluation by means of several diagnostic techniques. This case also stresses the phenotypic heterogeneity of the cytoskeleton of malignant melanomas and therefore their varied immunomorphologic characteristics.     

肺癌及肺良性病变角蛋白和AgNOR检测分析

目的：探讨肺良恶性病变中角蛋白和核仁组织区的表达意义。方法：采用免疫组化ＡＢＣ法和ＡｇＮＯＲ染色，对５４例肺良恶性病变进行检测。结果：角蛋白表达在低分化腺癌最高，ＡｇＮＯＲ表达以未分化癌最高。结论：角蛋白对判定肺癌类型有一定帮助，ＡｇＮＯＲ颗粒数目对判定肺癌的分化程度和预后有重要意义。角蛋白和ＡｇＮＯＲ对肺癌的类型鉴别及预后判定有重要作用。     

Analysis of thymic epithelial cell proliferation in vitro by combining bromodeoxyuridine and keratin labeling in an immunofluorescence assay

A simple method of analyzing thymic epithelial cell (TEC) proliferation has been developed by combining bromodeoxyuridine (BrDU) and keratin labeling in an immunofluorescence assay. The first reagent specifically visualizes the cells entering the S phase of the cell cycle, whereas the second immunostaining reveals which of the proliferating BrDU-positive cells actually belong to the epithelial lineage. This method, besides being rapid and free of radioactivity, appears to be reliable in view of the minor variations in the percentages of BrDU+ TEC observed in several distinct experiments. Thus, BrDU/keratin immunolabeling appears to represent a useful tool for the analysis of in vitro TEC proliferation.