Yu Lin, Daniels Joseph P, Wu Huihui, Wolf Matthew J
Department of Medicine, Duke University Medical Center, Durham, NC 27710, USA.
Sci Signal. 2015 Feb 3;8(362):ra13. doi: 10.1126/scisignal.2005719.
Organ hypertrophy can result from enlargement of individual cells or from cell proliferation or both. Activating mutations in the serine-threonine kinase Raf cause cardiac hypertrophy and contribute to Noonan syndrome in humans. Cardiac-specific expression of activated Raf also causes hypertrophy in Drosophila melanogaster. We found that Yorkie (Yki), a transcriptional coactivator in the Hippo pathway that regulates organ size, is required for Raf-induced cardiac hypertrophy in flies. Although aberrant activation of Yki orthologs stimulates cardiac hyperplasia in mice, cardiac-specific expression of an activated mutant form of Yki in fruit flies caused cardiac hypertrophy without hyperplasia. Knockdown of Yki caused cardiac dilation without loss of cardiomyocytes and prevented Raf-induced cardiac hypertrophy. In flies, Yki-induced cardiac hypertrophy required the TEA domain-containing transcription factor Scalloped, and, in mammalian cells, expression of mouse Raf(L613V), an activated form of Raf with a Noonan syndrome mutation, increased Yki-induced Scalloped activity. Furthermore, overexpression of Tgi (a Tondu domain-containing Scalloped-binding corepressor) in the fly heart abrogated Yki- or Raf-induced cardiac hypertrophy. Thus, crosstalk between Raf and Yki occurs in the heart and can influence Raf-mediated cardiac hypertrophy.
器官肥大可由单个细胞的增大、细胞增殖或两者共同作用引起。丝氨酸 - 苏氨酸激酶Raf中的激活突变会导致心脏肥大,并与人类的努南综合征有关。激活的Raf在心脏中的特异性表达也会导致黑腹果蝇出现肥大。我们发现,Yorkie(Yki)是一种在调节器官大小的Hippo信号通路中的转录共激活因子,是果蝇中Raf诱导的心脏肥大所必需的。虽然Yki直系同源物的异常激活会刺激小鼠心脏增生,但在果蝇中,激活突变形式的Yki在心脏中的特异性表达会导致心脏肥大而无增生。敲低Yki会导致心脏扩张而不损失心肌细胞,并能阻止Raf诱导的心脏肥大。在果蝇中,Yki诱导的心脏肥大需要含TEA结构域的转录因子Scalloped,并且在哺乳动物细胞中,具有努南综合征突变的激活形式的Raf即小鼠Raf(L613V)的表达会增加Yki诱导的Scalloped活性。此外,在果蝇心脏中过表达Tgi(一种含Tondu结构域的Scalloped结合共抑制因子)可消除Yki或Raf诱导的心脏肥大。因此,Raf和Yki之间的相互作用发生在心脏中,并会影响Raf介导的心脏肥大。