D'Amico Agata Grazia, Maugeri Grazia, Reitano Rita, Cavallaro Sebastiano, D'Agata Velia
San Raffaele Open University of Rome, Rome, Italy.
Section of Human Anatomy and Histology, Department of Biomedical and Biotechnological Sciences, University of Catania, Via S.Sofia, 87, 95123, Catania, Italy.
Protein J. 2016 Oct;35(5):354-362. doi: 10.1007/s10930-016-9679-5.
PARK2 gene's mutations are related to the familial form of juvenile Parkinsonism, also known as the autosomic recessive juvenile Parkinsonism. This gene encodes for parkin, a 465-amino acid protein. To date, a large number of parkin isoforms, generated by an alternative splicing mechanism, have been described. Currently, Gene Bank lists 27 rat PARK2 transcripts, which matches to 20 exclusive parkin alternative splice variants. Despite the existence of these isoforms, most of the studies carried out so far, have been focused only on the originally cloned parkin. In this work we have analyzed the expression profile of parkin isoforms in some rat brain areas including prefrontal cortex, hippocampus, substantia nigra and cerebellum. To discriminate among these isoforms, we detected their localization through the use of two antibodies that are able to identify different domains of the parkin canonical sequence. Our analysis has revealed that at least fourteen parkin isoforms are expressed in rat brain with a various distribution in the regions analyzed. Our study might help to elucidate the pathophysiological role of these proteins in the central nervous system.
PARK2基因的突变与青少年帕金森病的家族形式有关，也称为常染色体隐性青少年帕金森病。该基因编码一种由465个氨基酸组成的蛋白质——帕金蛋白。迄今为止，已经描述了大量由可变剪接机制产生的帕金蛋白异构体。目前，基因库列出了27种大鼠PARK2转录本，它们与20种独特的帕金蛋白可变剪接变体相对应。尽管存在这些异构体，但迄今为止进行的大多数研究仅集中在最初克隆的帕金蛋白上。在这项工作中，我们分析了帕金蛋白异构体在大鼠的一些脑区中的表达谱，这些脑区包括前额叶皮质、海马体、黑质和小脑。为了区分这些异构体，我们通过使用两种能够识别帕金蛋白标准序列不同结构域的抗体来检测它们的定位。我们的分析表明，至少有14种帕金蛋白异构体在大鼠脑中表达，并且在所分析的区域中分布各异。我们的研究可能有助于阐明这些蛋白质在中枢神经系统中的病理生理作用。
