Wheat Kernel Ingestion Protects from Progression of Muscle Weakness in mdx Mice, an Animal Model of Duchenne Muscular Dystrophy

Hübner, Christoph; Lehr, Hans‐Anton; Bodlaj, Robert; Finckh, Barbara; Oexle, Konrad; Marklund, Stefan L.; Freudenberg, Klaus; Kontush, Anatol; Speer, Astrid; Terwolbeck, Klaus; Voït, Thomas; Kohlschütter, Alfried

doi:10.1203/00006450-199609000-00013

Cited by 21 publications

(9 citation statements)

References 40 publications

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“…One obvious reference can be made to the importance of α-tropomyosin in the polymerisation of actin and in the mitotic cycle. Moreover, a role of α-tocopherol in the maintenance of muscular structure and function has been suggested and a therapeutic benefit in several muscular dystrophies has been obtained with vitamin E [11,[21][22][23][24][25]. These problems are currently under investigation.…”

Section: Discussionmentioning

confidence: 99%

Molecular basis of α‐tocopherol control of smooth muscle cell proliferation

et al. 1998

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Rat and human vascular smooth muscle cell proliferation is specifically sensitive to alpha-tocopherol, but not beta-tocopherol. The former, but not the latter, is capable of limiting proliferation and inhibiting protein kinase C activity in a dose-dependent manner. The phenomenon occurs at concentrations in the range 10-50 microM. beta-tocopherol addition together with alpha-tocopherol, prevents both cell growth and protein kinase C inhibition. alpha-tocopherol increases de novo synthesis of protein kinase C molecules. The enzyme specific activity, however, is diminished, due to a decreased phosphorylation of protein kinase C, occurring in the presence of alpha-tocopherol. Experiments with protein kinase C isoform-specific inhibitors and precipitating antibodies show that the only isoform affected by alpha-tocopherol is protein kinase C-alpha. The effect of alpha-tocopherol is prevented by okadaic acid indicating a phosphatase of the PP2A type as responsible for protein kinase C-alpha dephosphorylation produced in the presence of alpha-tocopherol. At a gene level alpha-tocopherol but not beta-tocopherol induces a transient activation of alpha-tropomyosin gene transcription and protein expression. It is proposed that, by inhibiting protein kinase C activity via an activation of a phosphatase PP2A, alpha-tocopherol controls smooth muscle cell proliferation through changes in gene expression.

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Section: Discussionmentioning

confidence: 99%

Molecular basis of α‐tocopherol control of smooth muscle cell proliferation

et al. 1998

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“…Cage bedding should also be considered as some bedding (e.g. wheat kernels) contains high levels of Vitamin E (anti-oxidant) and can have effects on mdx pathology [13]. Shell grit bedding or dried corn cob with nothing added is recommended.…”

Section: Methodological Standardsmentioning

confidence: 99%

Enhancing translation: Guidelines for standard pre-clinical experiments in mdx mice

Willmann

Benatar

et al. 2012

Neuromuscular Disorders

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Duchenne Muscular Dystrophy is an X-linked disorder that affects boys and leads to muscle wasting and death due to cardiac involvement and respiratory complications. The cause is the absence of dystrophin, a large structural protein indispensable for muscle cell function and viability. Neither an effective treatment nor a cure is available at the present time. The mdx mouse has become the standard animal model for pre-clinical evaluation of potential therapeutic treatments. Recent years have seen a rapid increase in the number of experimental compounds being evaluated in the mdx mouse. There is, however, much variability in the design of these pre-clinical experimental studies. This has made it difficult to interpret and compare published data from different laboratories and to evaluate the potential of a treatment for application to patients. The authors therefore propose the introduction of a standard study design for the mdx mouse model. Several aspects, including animal care, sampling times and choice of tissues, as well as recommended endpoints and methodologies are addressed and, for each aspect, a standard procedure is proposed. Testing of all new molecules/drugs using a widely accepted and agreed upon standard experimental protocol would greatly improve the power of pre-clinical experimentations and help identifying promising therapies for the translation into clinical trials for boys with Duchenne Muscular Dystrophy.

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“…The disease is caused by a defect in the gene encoding dystrophin, a protein located on the inner surface of the plasma membrane (44). The exact function of dystrophin is unknown, and the prospects for successful treatment of DMD remain uncertain, although numerous studies of gene therapy for DMD (21,22,42,43) and of pharmacological treatment (8,17,23,33,37,40,46) have been published.…”

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confidence: 99%

Dietary NaCl supplementation prevents muscle necrosis in a mouse model of Duchenne muscular dystrophy

Yoshida

Yonetani²,

Shirasaki³

et al. 2006

American Journal of Physiology-Regulatory, Integrative and Comp

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The mdx mouse is an animal model for Duchenne muscular dystrophy. Mdx mice fed a 12% NaCl diet from birth up to 20 days of age (mdx-Na mice) had an approximately 50% reduction in serum creatine kinase (CK) activity compared with mdx mice fed a standard diet. Most notably, necrotic fibers in tibialis anterior (TA) muscle of mdx-Na mice were reduced by 99% and were similar in control mice. These mdx mice displayed significantly elevated blood Ca2+ and Na+ levels, while the total calcium content of their TA muscle was reduced to the level of control mice. In addition, mdx-Na mice had elevated zinc and magnesium contents in their TA muscle. These results suggest that elevated serum Na+ leads to Ca2+ extrusion from muscle via the Na+/Ca2+ exchanger causing a decrease in intracellular Ca2+ levels and an increase in blood Ca2+ levels. Extracellular Ca2+ and, in addition, Zn2+ and Mg2+ might also contribute to the stabilization of the cell membrane. Other possibilities explaining the surprisingly efficacious beneficial effect of dietary sodium exist and are discussed.

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Wheat Kernel Ingestion Protects from Progression of Muscle Weakness in mdx Mice, an Animal Model of Duchenne Muscular Dystrophy

Cited by 21 publications

References 40 publications

Molecular basis of α‐tocopherol control of smooth muscle cell proliferation

Molecular basis of α‐tocopherol control of smooth muscle cell proliferation

Enhancing translation: Guidelines for standard pre-clinical experiments in mdx mice

Dietary NaCl supplementation prevents muscle necrosis in a mouse model of Duchenne muscular dystrophy

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