Gundersen K, Rabben I, Klocke B J, Merlie J P
Department of Neurophysiology, University of Oslo, Blindern, Norway.
Mol Cell Biol. 1995 Dec;15(12):7127-34. doi: 10.1128/MCB.15.12.7127.
To investigate the role of myogenin in regulating acetylcholine receptor expression in adult muscle, this muscle-specific basic helix-loop-helix transcription factor was overexpressed in transgenic mice by using regulatory elements conferring strong expression confined to differentiated postmitotic muscle fibers. Many of the transgenic mice died during the first postnatal week, but those that survived into adulthood displayed normal muscle histology, gross morphology, and motor behavior. The mRNA levels of all five acetylcholine receptor subunits (alpha, beta, gamma, delta, and epsilon) were, however, elevated. Also, the level of receptor protein was increased and high levels of receptors were present throughout the extrasynaptic surface membrane of the muscle fibers. Thus, elevated levels of myogenin are apparently sufficient to induce acetylcholine supersensitivity in normally innervated muscle of adult mice. The high neonatal mortality rate of the mice overexpressing myogenin hindered the propagation of a stable line. In an attempt to increase survival, myogenin overexpressers were mated with a line of transgenic mice overexpressing Id-1, a negative regulator that interacts with the basic helix-loop-helix family of transcription factors. The Id-1 transgene apparently worked as a second site suppressor and abolished the high rate of neonatal mortality. This effect indicates that Id-1 and myogenin interact directly or indirectly in these animals. Further study indicated that myogenin overexpression had no effect on the level of endogenous myogenin mRNA, while the levels of myoD and MRF4 mRNAs were reduced. Overexpression of the negative regulator Id-1 increased the mRNA levels of all the myogenic factors. These findings are consistent with a hypothesis suggesting that myogenic factors are influenced by mechanisms that maintain cellular homeostasis.
为了研究肌细胞生成素在调节成年肌肉中乙酰胆碱受体表达方面的作用,通过使用赋予强表达的调控元件,使这种肌肉特异性的碱性螺旋-环-螺旋转录因子在转基因小鼠中过表达,该调控元件可将表达限制在分化后的有丝分裂后肌纤维中。许多转基因小鼠在出生后的第一周内死亡,但那些存活到成年的小鼠表现出正常的肌肉组织学、大体形态和运动行为。然而,所有五个乙酰胆碱受体亚基(α、β、γ、δ和ε)的mRNA水平都升高了。此外,受体蛋白水平增加,并且在肌纤维的整个突触外表面膜上都存在高水平的受体。因此,肌细胞生成素水平的升高显然足以在成年小鼠正常支配的肌肉中诱导乙酰胆碱超敏反应。过表达肌细胞生成素的小鼠的高新生儿死亡率阻碍了稳定品系的繁殖。为了提高存活率,将过表达肌细胞生成素的小鼠与过表达Id-1的转基因小鼠品系交配,Id-1是一种与转录因子的碱性螺旋-环-螺旋家族相互作用的负调节因子。Id-1转基因显然起到了第二位点抑制作用,并消除了高新生儿死亡率。这种效应表明Id-1和肌细胞生成素在这些动物中直接或间接相互作用。进一步的研究表明,肌细胞生成素的过表达对内源性肌细胞生成素mRNA水平没有影响,而肌分化蛋白(MyoD)和肌肉调节因子4(MRF4)mRNA的水平降低。负调节因子Id-1的过表达增加了所有生肌因子的mRNA水平。这些发现与一个假设一致,该假设认为生肌因子受维持细胞稳态机制的影响。