Ploper Diego, Taelman Vincent F, Robert Lidia, Perez Brian S, Titz Björn, Chen Hsiao-Wang, Graeber Thomas G, von Euw Erika, Ribas Antoni, De Robertis Edward M
Howard Hughes Medical Institute and Department of Biological Chemistry.
Department of Medicine, Division of Hematology-Oncology.
Proc Natl Acad Sci U S A. 2015 Feb 3;112(5):E420-9. doi: 10.1073/pnas.1424576112. Epub 2015 Jan 20.
Canonical Wnt signaling plays an important role in development and disease, regulating transcription of target genes and stabilizing many proteins phosphorylated by glycogen synthase kinase 3 (GSK3). We observed that the MiT family of transcription factors, which includes the melanoma oncogene MITF (micropthalmia-associated transcription factor) and the lysosomal master regulator TFEB, had the highest phylogenetic conservation of three consecutive putative GSK3 phosphorylation sites in animal proteomes. This finding prompted us to examine the relationship between MITF, endolysosomal biogenesis, and Wnt signaling. Here we report that MITF expression levels correlated with the expression of a large subset of lysosomal genes in melanoma cell lines. MITF expression in the tetracycline-inducible C32 melanoma model caused a marked increase in vesicular structures, and increased expression of late endosomal proteins, such as Rab7, LAMP1, and CD63. These late endosomes were not functional lysosomes as they were less active in proteolysis, yet were able to concentrate Axin1, phospho-LRP6, phospho-β-catenin, and GSK3 in the presence of Wnt ligands. This relocalization significantly enhanced Wnt signaling by increasing the number of multivesicular bodies into which the Wnt signalosome/destruction complex becomes localized upon Wnt signaling. We also show that the MITF protein was stabilized by Wnt signaling, through the novel C-terminal GSK3 phosphorylations identified here. MITF stabilization caused an increase in multivesicular body biosynthesis, which in turn increased Wnt signaling, generating a positive-feedback loop that may function during the proliferative stages of melanoma. The results underscore the importance of misregulated endolysosomal biogenesis in Wnt signaling and cancer.
经典Wnt信号通路在发育和疾病中发挥重要作用,可调节靶基因转录并稳定许多被糖原合酶激酶3(GSK3)磷酸化的蛋白质。我们观察到,MiT转录因子家族,包括黑色素瘤癌基因MITF(小眼相关转录因子)和溶酶体主要调节因子TFEB,在动物蛋白质组中具有三个连续假定GSK3磷酸化位点的最高系统发育保守性。这一发现促使我们研究MITF、内溶酶体生物发生和Wnt信号通路之间的关系。在此我们报告,MITF表达水平与黑色素瘤细胞系中一大类溶酶体基因的表达相关。在四环素诱导的C32黑色素瘤模型中,MITF表达导致囊泡结构显著增加,并增加了晚期内体蛋白(如Rab7、LAMP1和CD63)的表达。这些晚期内体不是功能性溶酶体,因为它们在蛋白水解中活性较低,但在存在Wnt配体的情况下能够聚集Axin1、磷酸化LRP6、磷酸化β-连环蛋白和GSK3。这种重新定位通过增加多泡体的数量显著增强了Wnt信号通路,Wnt信号小体/破坏复合物在Wnt信号传导时会定位于多泡体中。我们还表明,通过此处鉴定的新型C末端GSK3磷酸化,Wnt信号通路可使MITF蛋白稳定。MITF的稳定导致多泡体生物合成增加,进而增加Wnt信号通路,产生一个可能在黑色素瘤增殖阶段起作用的正反馈环。结果强调了内溶酶体生物发生失调在Wnt信号通路和癌症中的重要性。