Department of Oncologic Sciences, Mitchell Cancer Institute, University of South Alabama, Mobile, AL, USA.
Exp Cell Res. 2012 Jun 10;318(10):1086-93. doi: 10.1016/j.yexcr.2012.03.024. Epub 2012 Apr 3.
HSP40 family member MRJ (DNAJB6) has been in the spot light for its relevance to Huntington's, Parkinson's diseases, limb-girdle muscular dystrophy, placental development, neural stem cells, cell cycle and malignancies such as breast cancer and melanoma. This gene has two spliced variants coding for 2 distinct proteins with significant homology. However, MRJ(L) (large variant) is predominantly localized to the nucleus whereas MRJ(S) (small variant) is predominantly cytoplasmic. Interestingly MRJ(S) translocates to the nucleus in response to heat shock. The classical heat shock proteins respond to crises (stress) by increasing the number of molecules, usually by transcriptional up-regulation. Our studies imply that a quick increase in the molar concentration of MRJ in the nuclear compartment is a novel method by which MRJ responds to stress. We found that MRJ(S) shows NLS (nuclear localization signal) independent nuclear localization in response to heat shock and hypoxia. The specificity of this response is realized due to lack of such response by MRJ(S) when challenged by other stressors, such as some cytokines or UV light. Deletion analysis has allowed us to narrow down on a 20 amino acid stretch at the C-terminal region of MRJ(S) as a potential stress sensing region. Functional studies indicated that constitutive nuclear localization of MRJ(S) promoted attributes of malignancy such as proliferation and invasiveness overall indicating distinct phenotypic characteristics of nuclear MRJ(S).
热休克蛋白 40 家族成员 MRJ(DNAJB6)因其与亨廷顿病、帕金森病、肢带型肌营养不良症、胎盘发育、神经干细胞、细胞周期以及乳腺癌和黑色素瘤等恶性肿瘤的相关性而备受关注。该基因有两个剪接变异体,编码两种具有显著同源性的不同蛋白质。然而,MRJ(L)(大变异体)主要定位于细胞核,而 MRJ(S)(小变异体)主要定位于细胞质。有趣的是,MRJ(S)在受热休克时会向核内移位。经典的热休克蛋白通过增加分子数量来应对危机(应激),通常通过转录上调。我们的研究表明,MRJ 在核区摩尔浓度的快速增加是 MRJ 应对应激的一种新方法。我们发现,MRJ(S)在受热休克和缺氧时表现出不依赖核定位信号(NLS)的核定位。这种反应的特异性是由于 MRJ(S)在受到其他应激源(如某些细胞因子或紫外线)挑战时没有这种反应而实现的。缺失分析使我们能够将 MRJ(S)的 C 末端区域的 20 个氨基酸延伸确定为潜在的应激感应区域。功能研究表明,MRJ(S)的组成性核定位促进了增殖和侵袭等恶性特征,总体上表明核 MRJ(S)具有独特的表型特征。