Micaroni Massimo, Malquori Lorenzo
School of Biosciences, University of Exeter, Exeter, England EX4 4QD, UK.
Int J Mol Sci. 2013 Jan 4;14(1):674-83. doi: 10.3390/ijms14010674.
The ATP2C1 gene encodes for the secretory pathway calcium (Ca2+)-ATPase pump (SPCA1), which localizes along the secretory pathway, mainly in the trans-Golgi. The loss of one ATP2C1 allele causes Hailey-Hailey disease in humans but not mice. Examining differences in genomic organization between mouse and human we speculate that the overlap between ATP2C1 and ASTE1 genes only in humans could explain this different response to ATP2C1 dysregulation. We propose that ASTE1, overlapping with ATP2C1 in humans, affects alternative splicing, and potentially protein expression of the latter. If dysregulated, the composition of the SPCA1 isoform pool could diverge from the physiological status, affecting cytosolic Ca2+-signaling, and in turn perturbing cell division, leading to cell death or to neoplastic transformation.
ATP2C1基因编码分泌途径钙(Ca2+)-ATP酶泵(SPCA1),该泵定位于分泌途径,主要位于反式高尔基体。一个ATP2C1等位基因的缺失会导致人类患黑利-黑利病,但不会导致小鼠患病。通过研究小鼠和人类基因组组织的差异,我们推测仅在人类中ATP2C1和ASTE1基因的重叠可以解释对ATP2C1失调的这种不同反应。我们提出,在人类中与ATP2C1重叠的ASTE1会影响可变剪接,并可能影响后者的蛋白质表达。如果失调,SPCA1同工型库的组成可能会偏离生理状态,影响胞质Ca2+信号传导,进而扰乱细胞分裂,导致细胞死亡或肿瘤转化。