Genetic Background Affects Properties of Satellite Cells and mdx Phenotypes

Fukada, So‐ichiro; Morikawa, Daisuke; Yamamoto, Yoshiyuki; Yoshida, Tokuyuki; Sumie, Noriaki; Yamaguchi, Masahiko; Ito, Takahito; Miyagoe-Suzuki, Yuko; Takeda, Shin’ichi; Tsujikawa, Kazutake; Yamamoto, Hiroshi

doi:10.2353/ajpath.2010.090887

Cited by 158 publications

(198 citation statements)

References 44 publications

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“…Ten measurements were recorded, which were separated by 10‐min rest periods. The two highest and two lowest values were discarded from these measurements, and the remaining six values were averaged (Fukada et al., 2010). …”

Section: Methodsmentioning

confidence: 99%

Role of dietary amino acid balance in diet restriction‐mediated lifespan extension, renoprotection, and muscle weakness in aged mice

et al. 2018

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SummaryExtending healthy lifespan is an emerging issue in an aging society. This study was designed to identify a dietary method of extending lifespan, promoting renoprotection, and preventing muscle weakness in aged mice, with a focus on the importance of the balance between dietary essential (EAAs) and nonessential amino acids (NEAAs) on the dietary restriction (DR)‐induced antiaging effect. Groups of aged mice were fed ad libitum, a simple DR, or a DR with recovering NEAAs or EAAs. Simple DR significantly extended lifespan and ameliorated age‐related kidney injury; however, the beneficial effects of DR were canceled by recovering dietary EAA but not NEAA. Simple DR prevented the age‐dependent decrease in slow‐twitch muscle fiber function but reduced absolute fast‐twitch muscle fiber function. DR‐induced fast‐twitch muscle fiber dysfunction was improved by recovering either dietary NEAAs or EAAs. In the ad libitum‐fed and the DR plus EAA groups, the renal content of methionine, an EAA, was significantly higher, accompanied by lower renal production of hydrogen sulfide (H2S), an endogenous antioxidant. Finally, removal of methionine from the dietary EAA supplement diminished the adverse effects of dietary EAA on lifespan and kidney injury in the diet‐restricted aged mice, which were accompanied by a recovery in H2S production capacity and lower oxidative stress. These data imply that a dietary approach could combat kidney aging and prolong lifespan, while preventing muscle weakness, and suggest that renal methionine metabolism and the trans‐sulfuration pathway could be therapeutic targets for preventing kidney aging and subsequently promoting healthy aging.

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Section: Methodsmentioning

confidence: 99%

Role of dietary amino acid balance in diet restriction‐mediated lifespan extension, renoprotection, and muscle weakness in aged mice

et al. 2018

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“…The D2 strain was previously noted to disrupt myoblast fusion in the mdx mouse model, and there are other QTLs that modify muscular dystrophy in the D2 strain (31,32). Membrane repair and membrane fusion share some of the same molecular machinery (33).…”

Section: Discussionmentioning

confidence: 99%

Annexin A6 modifies muscular dystrophy by mediating sarcolemmal repair

Swaggart¹,

Demonbreun²,

et al. 2014

Proc. Natl. Acad. Sci. U.S.A.

123

161

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Many monogenic disorders, including the muscular dystrophies, display phenotypic variability despite the same disease-causing mutation. To identify genetic modifiers of muscular dystrophy and its associated cardiomyopathy, we used quantitative trait locus mapping and whole genome sequencing in a mouse model. This approach uncovered a modifier locus on chromosome 11 associated with sarcolemmal membrane damage and heart mass. Whole genome and RNA sequencing identified Anxa6, encoding annexin A6, as a modifier gene. A synonymous variant in exon 11 creates a cryptic splice donor, resulting in a truncated annexin A6 protein called ANXA6N32. Live cell imaging showed that annexin A6 orchestrates a repair zone and cap at the site of membrane disruption. In contrast, ANXA6N32 dramatically disrupted the annexin A6-rich cap and the associated repair zone, permitting membrane leak. Anxa6 is a modifier of muscular dystrophy and membrane repair after injury.dystrophin | muscle | plasma membrane

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“…In line with this rationale, Fukada and colleagues found that mice of the DBA/2 genetic background displayed impaired muscle regeneration and satellite cell self-renewal compared to other mouse strains [28]. They generated DBA/2- mdx mice and reported reduced muscle weight, fewer muscle fibers, increased fibrogenic and adipogenic accumulation and greater muscle weakness compared to the commonly used mdx mice of the C57BL/10 background.…”

Section: Dmd Is Also a Stem Cell Diseasementioning

confidence: 99%

Satellite Cells in Muscular Dystrophy – Lost in Polarity

Chang

Chevalier

Rudnicki

2016

Trends in Molecular Medicine

160

161

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Recent findings employing the mdx mouse model for Duchenne muscular dystrophy (DMD) have revealed that muscle satellite stem cells play a direct role in contributing to disease etiology and progression of DMD, the most common and severe form of muscular dystrophy. Lack of dystrophin expression in DMD has critical consequences in satellite cells including an inability to establish cell polarity, abrogation of asymmetric satellite stem cell divisions and failure to enter the myogenic program. Thus, muscle wasting in dystrophic mice is not only caused by myofiber fragility, but is exacerbated by intrinsic satellite cell dysfunction leading to impaired regeneration. Despite intense research and clinical efforts, there is still no effective cure for DMD. In this review, we highlight recent research advances in DMD and discuss the current state of treatment and importantly, how we can incorporate satellite cell-targeted therapeutic strategies to correct satellite cell dysfunction in DMD.

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Genetic Background Affects Properties of Satellite Cells and mdx Phenotypes

Cited by 158 publications

References 44 publications

Role of dietary amino acid balance in diet restriction‐mediated lifespan extension, renoprotection, and muscle weakness in aged mice

Role of dietary amino acid balance in diet restriction‐mediated lifespan extension, renoprotection, and muscle weakness in aged mice

Annexin A6 modifies muscular dystrophy by mediating sarcolemmal repair

Satellite Cells in Muscular Dystrophy – Lost in Polarity

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