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Open AccessJournal ArticleDOI

Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy

TLDR
A wide range of techniques used to measure parameters of muscle pathology in mdx mice are described and some basic techniques that might comprise standardised approaches for evaluation are identified.
About
This article is published in Neurobiology of Disease.The article was published on 2008-07-01 and is currently open access. It has received 301 citations till now. The article focuses on the topics: mdx mouse & Duchenne muscular dystrophy.

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Citations
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Threats to validity in the design and conduct of preclinical efficacy studies: a systematic review of guidelines for in vivo animal experiments

TL;DR: A systematic review of preclinical research guidelines and organized recommendations according to the type of validity threat (internal, construct, or external) or programmatic research activity they primarily address provided a starting point for developing preclinical guidelines in other disease domains.
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Phosphatidylserine receptor BAI1 and apoptotic cells as new promoters of myoblast fusion.

TL;DR: Data are identified to identify apoptotic cells as a new type of cue that induces signalling via the phosphatidylserine receptor BAI1 to promote fusion of healthy myoblasts, with important implications for muscle development and repair.
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Injury-Induced Senescence Enables In Vivo Reprogramming in Skeletal Muscle

TL;DR: It is shown that acute and chronic injury enables transcription-factor-mediated reprogramming in skeletal muscle and this response frequently originates from Pax7+ muscle stem cells, highlighting a beneficial paracrine effect of injury-induced senescence on cellular plasticity.
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Mammalian animal models for Duchenne muscular dystrophy

TL;DR: The currently used mammalian animal models for DMD are compared with the aim of selecting and recommending the most appropriate ones for preclinical efficacy testing of new therapeutic strategies.
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Taurine: the appeal of a safe amino acid for skeletal muscle disorders

TL;DR: Taurine treatment can be beneficial to reduce sarcolemmal hyper-excitability in myotonia-related syndromes and may represent a guide-line for designing specific studies in patients of neuromuscular diseases.
References
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Journal ArticleDOI

Epigenetic programming by maternal behavior.

TL;DR: It is shown that an epigenomic state of a gene can be established through behavioral programming, and it is potentially reversible, suggesting a causal relation among epigenomicState, GR expression and the maternal effect on stress responses in the offspring.
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Environmental epigenomics and disease susceptibility.

TL;DR: An increasing body of evidence from animal studies supports the role of environmental epigenetics in disease susceptibility and recent studies have demonstrated for the first time that heritable environmentally induced epigenetic modifications underlie reversible transgenerational alterations in phenotype.
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Nongenomic Transmission Across Generations of Maternal Behavior and Stress Responses in the Rat

TL;DR: Results of cross-fostering studies reported here indicate that variations in maternal care can serve as the basis for a nongenomic behavioral transmission of individual differences in stress reactivity across generations.
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X chromosome-linked muscular dystrophy (mdx) in the mouse.

TL;DR: Linkage analysis with four X chromosome loci indicates that mdx maps in the Hq Bpa region of the mouse X chromosome, which gives a gene order of mdx-Tfm-Pgk-1-Ags, the same as for the equivalent genes on the human X chromosome.
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Molecular diversity of myofibrillar proteins: gene regulation and functional significance

TL;DR: The pattern of isogene expression varies during muscle development in relation to the different origin of myogenic cells and primary/secondary fiber generations and is affected by neural and hormonal influences.
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