文章：

Waldenström的巨球蛋白血症含有一种独特的蛋白质组，与其他b淋巴细胞增殖性疾病相比，Ku70严重表达不足

Waldenström’s macroglobulinemia harbors a unique proteome where Ku70 is severely underexpressed as compared with other B-lymphoproliferative disorders

原文发布日期：2012-09-07

DOI: 10.1038/bcj.2012.35

类型: Original Article

开放获取: 是

英文摘要：

Waldenström’s macroglobulinemia (WM) is a clonal B-cell lymphoproliferative disorder (LPD) of post-germinal center nature. Despite the fact that the precise molecular pathway(s) leading to WM remain(s) to be elucidated, a hallmark of the disease is the absence of the immunoglobulin heavy chain class switch recombination. Using two-dimensional gel electrophoresis, we compared proteomic profiles of WM cells with that of other LPDs. We were able to demonstrate that WM constitutes a unique proteomic entity as compared with chronic lymphocytic leukemia and marginal zone lymphoma. Statistical comparisons of protein expression levels revealed that a few proteins are distinctly expressed in WM in comparison with other LPDs. In particular we observed a major downregulation of the double strand repair protein Ku70 (XRCC6); confirmed at both the protein and RNA levels in an independent cohort of patients. Hence, we define a distinctive proteomic profile for WM where the downregulation of Ku70—a component of the non homologous end-joining pathway—might be relevant in disease pathophysiology.

摘要翻译： 

瓦尔登斯特伦巨球蛋白血症（WM）是一种生发中心后来源的克隆性B细胞淋巴增殖性疾病。尽管导致WM的确切分子通路仍有待阐明，但该疾病的一个典型特征是免疫球蛋白重链类别转换重组缺失。通过采用双向凝胶电泳技术，我们比较了WM细胞与其他淋巴增殖性疾病的蛋白质组谱。我们证实，与慢性淋巴细胞白血病和边缘区淋巴瘤相比，WM构成了一个独特的蛋白质组实体。蛋白质表达水平的统计学比较显示，与其他淋巴增殖性疾病相比，WM中有少数蛋白质表达显著不同。我们特别观察到双链修复蛋白Ku70（XRCC6）的主要下调趋势，这一现象在独立患者队列中均已在蛋白质和RNA水平得到证实。因此，我们明确了WM独特的蛋白质组特征，其中非同源末端连接通路组分Ku70的下调可能与疾病病理生理机制相关。

原文链接：

Waldenström’s macroglobulinemia harbors a unique proteome where Ku70 is severely underexpressed as compared with other B-lymphoproliferative disorders