Novartis Institute for Biomedical Research, Cambridge, MA 02139, USA.
Curr Top Microbiol Immunol. 2010;346:267-78. doi: 10.1007/82_2010_78.
Activation of the PI3 kinase pathway can induce skeletal muscle hypertrophy, defined as an increase in skeletal muscle mass. In mammals, skeletal muscle hypertrophy occurs as a result of an increase in the size, as opposed to the number, of pre-existing skeletal muscle fibers. This pathway's effects on skeletal muscle have been implicated most prominently downstream of Insulin-like growth factor 1 signaling. IGF-1's pro-hypertrophy activity comes predominantly through its ability to activate the Phosphoinositide 3-kinase (PI3K)/Akt signaling pathway. Akt is a serine-threonine protein kinase that can induce protein synthesis and block the transcriptional upregulation of key mediators of skeletal muscle atrophy, the E3 ubiquitin ligases MuRF1 and MAFbx (also called Atrogin-1), by phosphorylating and thereby inhibiting the nuclear translocation of the FOXO (also called "forkhead") family of transcription factors. Once phosphorylated by Akt, the FOXOs are excluded from the nucleus, and upregulation of MuRF1 and MAFbx is blocked. MuRF1 and MAFbx mediate atrophy by ubiquitinating particular protein substrates, causing them to undergo degradation by the proteasome. MuRF1's substrates include several components of the sarcomeric thick filament, including Myosin Heavy Chain (MyHC). Thus, by blocking MuRF1 activation, IGF-1 helps prevent the breakdown of the thick filament under atrophy conditions.IGF1/PI3K/Akt signaling also can dominantly inhibit the effects of a secreted protein called "myostatin," which is a member of the TGFβ family of proteins. Deletion or inhibition of myostatin causes an increase in skeletal muscle size, because myostatin acts both to inhibit myoblast differentiation and to block the Akt pathway. Thus by blocking myostatin, PI3K/Akt activation stimulates differentiation and protein synthesis by this distinct mechanism. Myostatin induces the phosphorylation and activation of the transcription factors of Smad2 and Smad3, downstream of the ActRII (Activin Receptor type II)/Alk (Activin Receptor-like kinase) receptor complex. Other TGFβ-like molecules can also block differentiation, including TGF-b1, GDF-11, activinA, BMP-2 and BMP-7. As mentioned, myostatin also downregulates the Akt/mTOR/p70S6 protein synthesis pathway, which mediates both differentiation in myoblasts and hypertrophy in myotubes. Blockade of the Akt/mTOR pathway, using siRNA to RAPTOR, a component of "TORC1" (TOR signaling Complex 1), increases myostatin-induced phosphorylation of Smad2; this establishes a "feed-forward mechanism," because myostatin can downregulates TORC1, and this downregulation in turn amplifies myostatin signaling. Blockade of RAPTOR also facilitates myostatin's inhibition of muscle differentiation. When added to post-differentiated myotubes, myostatin causes a decrease in their diameter - however, this does not happen through the normal "atrophy pathway." Rather than causing upregulation of the E3 ubiquitin ligases MuRF1 and MAFbx, previously shown to mediate skeletal muscle atrophy, myostatin decreases expression of these atrophy markers in differentiated myotubes, as well as other genes normally upregulated during differentiation, such as MyoD and myogenin. These findings show that myostatin signaling acts by blocking genes induced during differentiation, even in a myotube, as opposed to activating the distinct "atrophy program."
PI3 激酶途径的激活可诱导骨骼肌肥大,定义为骨骼肌质量增加。在哺乳动物中,骨骼肌肥大是由于现有骨骼肌纤维大小的增加,而不是数量的增加。该途径对骨骼肌的影响主要发生在胰岛素样生长因子 1 信号转导的下游。IGF-1 的促肥大活性主要来自其激活磷酸肌醇 3-激酶(PI3K)/Akt 信号通路的能力。Akt 是一种丝氨酸-苏氨酸蛋白激酶,可通过磷酸化并抑制 FOXO(也称为“叉头”)转录因子家族的核易位,从而诱导蛋白质合成并阻止关键肌肉萎缩介质的转录上调,E3 泛素连接酶 MuRF1 和 MAFbx(也称为 Atrogin-1)。磷酸化后,FOXO 被排除在细胞核之外,并且 MuRF1 和 MAFbx 的上调被阻止。MuRF1 和 MAFbx 通过泛素化特定的蛋白质底物来介导萎缩,导致它们被蛋白酶体降解。MuRF1 的底物包括肌球蛋白重链(MyHC)等肌节厚丝的几个成分。因此,通过阻断 MuRF1 的激活,IGF-1 有助于防止在萎缩条件下厚丝的分解。IGF1/PI3K/Akt 信号还可以显著抑制一种称为“肌肉生长抑制素”的分泌蛋白的作用,肌肉生长抑制素是 TGFβ 蛋白家族的成员。肌肉生长抑制素的缺失或抑制会导致骨骼肌大小增加,因为肌肉生长抑制素既能抑制成肌细胞分化,又能阻断 Akt 途径。因此,通过阻断肌肉生长抑制素,PI3K/Akt 激活通过这种独特的机制刺激分化和蛋白质合成。肌肉生长抑制素诱导转录因子 Smad2 和 Smad3 的磷酸化和激活,该途径是 ActRII(激活素受体 II)/Alk(激活素受体样激酶)受体复合物下游的。其他 TGFβ 样分子也可以阻断分化,包括 TGF-b1、GDF-11、激活素 A、BMP-2 和 BMP-7。如前所述,肌肉生长抑制素还下调 Akt/mTOR/p70S6 蛋白质合成途径,该途径介导成肌细胞的分化和肌管的肥大。使用 siRNA 阻断 Akt/mTOR 途径,靶向 RAPTOR,TORC1(TOR 信号复合物 1)的一个组成部分,增加了肌肉生长抑制素诱导的 Smad2 磷酸化;这建立了一个“反馈机制”,因为肌肉生长抑制素可以下调 TORC1,这种下调反过来又放大了肌肉生长抑制素信号。RAPTOR 的阻断也有利于肌肉生长抑制素抑制肌肉分化。当添加到分化后的肌管中时,肌肉生长抑制素会导致它们的直径减小——然而,这不是通过正常的“萎缩途径”发生的。肌肉生长抑制素不会引起 E3 泛素连接酶 MuRF1 和 MAFbx 的上调,这两种酶以前被证明介导骨骼肌萎缩,而是在分化的肌管中降低这些萎缩标志物的表达,以及其他在分化过程中通常上调的基因,如 MyoD 和 myogenin。这些发现表明,肌肉生长抑制素信号通过阻断分化过程中诱导的基因起作用,即使在肌管中也是如此,而不是激活独特的“萎缩程序”。
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