Christophi G P, Isackson P J, Blaber S, Blaber M, Rodriguez M, Scarisbrick I A
Department of Physical Medicine & Rehabilitation, Mayo Medical and Graduate Schools, Rochester, Minnesota, USA.
J Neurochem. 2004 Dec;91(6):1439-49. doi: 10.1111/j.1471-4159.2004.02826.x.
Kallikrein 6 is a serine protease expressed abundantly in normal adult human and rodent CNS, and therein is regulated by injury. In the case of CNS demyelinating disease, K6 expression in CNS occurs additionally in perivascular and parenchymal inflammatory cells suggesting a role in pathogenesis. Herein we describe two unique transcripts that occur within the human and mouse K6 genes that differ in their 5'-untranslated regions. These transcripts have identical translation initiation sites in exon 3, are expressed in a tissue-specific fashion and are differentially regulated in response to CNS injury. While the human and mouse 5'-transcripts differ in sequence they are identical in genomic organization and tissue-specific expression. The most 5'-transcript, designated transcript 1, includes exon 1-7, and was detectable in all CNS regions, but not in any non-CNS tissues examined (spleen, thymus, liver, kidney, pancreas, submandibular gland and peripheral nerve). In contrast, transcript 2 lacks exon 1, but contains a unique sequence at the 5'-end of exon 2, designated exon 2A. Transcript 2 was expressed both in CNS and in each peripheral tissue. In a murine model of human CNS demyelinating inflammatory disease induced by Theiler's picornovirus, mouse K6 transcript 1 was up-regulated in brain and spinal cord at acute and more chronic phases of CNS inflammation and demyelination, while overall transcript 2 expression was not significantly altered. However, in isolated splenocyte cultures, transcript 2 was up-regulated two-fold by cellular activation. Tissue-specific expression patterns and differential regulation in CNS disease indicates that each K6 5'-transcript is probably regulated by unique promoter elements and may serve as a molecular target to treat inflammatory demyelinating disease.
激肽释放酶6是一种丝氨酸蛋白酶,在正常成年人类和啮齿动物的中枢神经系统中大量表达,并在其中受损伤调节。在中枢神经系统脱髓鞘疾病中,中枢神经系统中的K6表达还出现在血管周围和实质炎症细胞中,提示其在发病机制中起作用。在此,我们描述了人类和小鼠K6基因中存在的两种独特转录本,它们在5'非翻译区有所不同。这些转录本在第3外显子中有相同的翻译起始位点,以组织特异性方式表达,并在中枢神经系统损伤时受到不同调节。虽然人类和小鼠的5'转录本在序列上不同,但它们在基因组组织和组织特异性表达方面是相同的。最靠近5'端的转录本,即转录本1,包含第1至7外显子,在所有中枢神经系统区域均可检测到,但在所检测(脾脏、胸腺、肝脏、肾脏、胰腺、下颌下腺和周围神经)的任何非中枢神经系统组织中均未检测到。相比之下,转录本2缺少第1外显子,但在第2外显子的5'端含有一个独特序列,称为第2A外显子。转录本2在中枢神经系统和每个外周组织中均有表达。在由泰勒氏微小核糖核酸病毒诱导的人类中枢神经系统脱髓鞘炎症性疾病的小鼠模型中,小鼠K6转录本1在中枢神经系统炎症和脱髓鞘的急性期和更慢性期在脑和脊髓中上调,而转录本2的总体表达没有显著改变。然而,在分离出的脾细胞培养物中,转录本2通过细胞激活上调了两倍。中枢神经系统疾病中的组织特异性表达模式和差异调节表明,每个K6 5'转录本可能受独特的启动子元件调节,并可能成为治疗炎症性脱髓鞘疾病的分子靶点。