Expression of Myostatin and Follistatin in<i>Mdx</i>Mice, an Animal Model for Muscular Dystrophy

Abe, Shinichi; Soejima, Masakazu; Iwanuma, Osamu; Saka, Hideki; Matsunaga, Satoru; Sakiyama, Kazuo; Ide, Yoshinobu

doi:10.2108/zsj.26.315

Cited by 28 publications

(23 citation statements)

References 28 publications

(32 reference statements)

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“…Follistatin mRNA expression increases gradually from 2 weeks, reaching maximum expression at 4 weeks in both muscles. Similar results were previously reported (Abe et al 2009a). TA muscle showed higher expression compared to masseter muscle, showing a significant difference at 3 and 4 weeks (Fig.…”

Section: Discussionsupporting

confidence: 91%

“…For this reason, we believe that myostatin could control the maturity of skeletal muscle. Comparing expression of myostatin and follistatin in the B10 mouse (control group) and mdx mouse (experimental group), higher expression was seen in the mdx mouse than in the B10 mouse at 2, 3 and 4 weeks (Abe et al 2009a). On the other hand, expression of myostatin, follistatin and decorin in both the experimental and control groups has been reported (Abe et al 2009a, b).…”

Section: Discussionmentioning

confidence: 99%

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A comparative study of myostatin, follistatin and decorin expression in muscle of different origin

et al. 2011

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Muscle regeneration supports muscle function in aging, and plays a role in the functional impairment caused by progressive neuromuscular diseases. Major substances controlling this process are growth factors and the extracellular matrix (ECM). Thus, follistatin is known to antagonize the function of several members of the TGF-β family of secreted signaling factors, including myostatin-the most powerful inhibitor of muscle growth characterized to date. Decorin-a small leucine-rich proteoglycan-traps myostatin and modulates its activity towards myogenic cells in the ECM. In addition, there are few reports concerning the regenerative muscle process of masseter muscles, which are of branchial arch origin, in mdx mice. Thus, in order to clarify the muscle regenerative process of masseter muscle, gene and protein expression of myostatin, follistatin and decorin were examined using the tibialis anterior (TA)muscle as a positive control. In both muscles, a gradual increase in mRNA myostatin, follistatin and decorin expression was detected, with the increase being greater in TA muscle than in masseter muscle. At 2 weeks, both muscles exhibited normal skeletal muscle cells. At 3 weeks, masseter muscle demonstrated scant areas of necrosis, whereas large necrotic zones were seen in TA muscle. At 4 weeks, the formation of necrotic tissue and presence of follistatin protein was observed clearly in masseter muscle. This result indicates that follistatin production is stimulated in the presence of necrosis. Interestingly, both muscles showed the same process of muscular formation, but with different time frames, which could be related to muscle origin.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

A comparative study of myostatin, follistatin and decorin expression in muscle of different origin

et al. 2011

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“…Additionally, we have verified that the size of L6E9 myotubes can be incremented through delivery of a dominant-negative ActRIIb form or increase of follistatin, confirming that multiple TGF- β regulators converge on ActRIIs receptors to determine the size of myofibers [19–21, 28, 29] and, importantly, that follistatin can neutralize most of them [19, 20, 22, 29, 30]. In addition to myostatin, GDF11, Activins (A and B) [29, 31], and BMP-9 and 10 proteins have been identified to bind ActRIIs [31].…”

Section: Discussionmentioning

confidence: 64%

L6E9 Myoblasts Are Deficient of Myostatin and Additional TGF-βMembers Are Candidates to Developmentally Control Their Fiber Formation

Rossi

Stoppani

Gobbo

et al. 2010

Journal of Biomedicine and Biotechnology

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This work provides evidence that the robust myoblast differentiation observed in L6E9 cells is causally linked to deficiency of myostatin, which, conversely, has been found to be expressed in C2C12 cells. However, despite the absence of endogenous myostatin, L6E9 myoblasts expressed functional Activin receptors type II (ActRIIs) and follistatin as well as the highly related TGF-β members Activins and GDF11, suggesting that in this cell line the regulation of fiber size might be under the control of multiple regulators regardless of myostatin. In line with this hypothesis, delivery of a dominant-negative ActRIIb form or the increase of follistatin, as obtained via Trichostatin treatment or stable transfection of a short human follistatin form, enhanced the L6E9 cell differentiation and further increased the size of myotubes, suggesting that L6E9 myoblasts provide a spontaneous myostatin knock-out in vitro model to study TGF-β ligands involved in developmental regulation of fiber size.

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“…Its expression was significantly higher in mdx mice than in wild-type mice 105. The inhibitory effect of myostatin on postnatal growth is mediated by its negative regulation of satellite cell activation, proliferation, and self-renewal106 as well as myoblast proliferation and differentiation 103,107…”

Section: Potential Drugs Targeting Inflammatory Mechanisms In Dmdmentioning

confidence: 99%

Anti-inflammatory drugs for Duchenne muscular dystrophy: focus on skeletal muscle-releasing factors

Miyatake

Shimizu‐Motohashi

Takeda

et al. 2016

DDDT

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Duchenne muscular dystrophy (DMD), an incurable and a progressive muscle wasting disease, is caused by the absence of dystrophin protein, leading to recurrent muscle fiber damage during contraction. The inflammatory response to fiber damage is a compelling candidate mechanism for disease exacerbation. The only established pharmacological treatment for DMD is corticosteroids to suppress muscle inflammation, however this treatment is limited by its insufficient therapeutic efficacy and considerable side effects. Recent reports show the therapeutic potential of inhibiting or enhancing pro- or anti-inflammatory factors released from DMD skeletal muscles, resulting in significant recovery from muscle atrophy and dysfunction. We discuss and review the recent findings of DMD inflammation and opportunities for drug development targeting specific releasing factors from skeletal muscles. It has been speculated that nonsteroidal anti-inflammatory drugs targeting specific inflammatory factors are more effective and have less side effects for DMD compared with steroidal drugs. For example, calcium channels, reactive oxygen species, and nuclear factor-κB signaling factors are the most promising targets as master regulators of inflammatory response in DMD skeletal muscles. If they are combined with an oligonucleotide-based exon skipping therapy to restore dystrophin expression, the anti-inflammatory drug therapies may address the present therapeutic limitation of low efficiency for DMD.

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Expression of Myostatin and Follistatin inMdxMice, an Animal Model for Muscular Dystrophy

Cited by 28 publications

References 28 publications

A comparative study of myostatin, follistatin and decorin expression in muscle of different origin

A comparative study of myostatin, follistatin and decorin expression in muscle of different origin

L6E9 Myoblasts Are Deficient of Myostatin and Additional TGF-βMembers Are Candidates to Developmentally Control Their Fiber Formation

Anti-inflammatory drugs for Duchenne muscular dystrophy: focus on skeletal muscle-releasing factors

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