Pre-clinical screening of drugs using the mdx mouse

Granchelli, Joseph A.; Pollina, Catherine M.; Hudecki, Michael S.

doi:10.1016/s0960-8966(99)00126-1

Cited by 144 publications

(163 citation statements)

References 26 publications

Supporting

Mentioning

146

Contrasting

Unclassified

Order By: Relevance

“…Although previous studies have observed benefits in whole-body strength with prednisone 19 and CrM 15 interventions alone, we did not detect consistently significant improvements with these individual treatments using peak grip strength in isolation. There are a number of different techniques and apparatuses currently available for measuring grip strength, 7,15,19 and often the strength superiority of a compound under investigation was reported at only one measurement time. 15,19 Recently, a number of grip strength testing factors were investigated, including operational parameters, and they substantially influenced test results.…”

Section: Discussionmentioning

confidence: 99%

“…Four dietary supplements (Table 1) were added in isolation and in combination (COMB) to the diets including (1) CrM (Creapure, CrM (n ϭ 10) mdx 2% CrM ϩ 1% soybean oil CLA (n ϭ 10) mdx 1% CLA HMB (n ϭ 10) mdx 0.5% HMB ϩ 1% soybean oil ␣-LA (n ϭ 10) mdx 0.5% ␣-LA ϩ 1% soybean oil COMB (n ϭ 10) mdx Combination therapy PRED (n ϭ 10) mdx methylprednisolone (2 mg/kg/d) COMB ϩ PRED (n ϭ 10) mdx Combination therapy ϩ methylprednisolone PLTR (n ϭ 10) 15 but most of the mdx mice experienced great difficulty and refused to run. The speed was subsequently decreased to 9.5 m/min for 25 min.…”

Section: Animalsmentioning

confidence: 99%

“…14 However, the superimposition of exercise in the mdx mouse results in progressive loss of limb muscle, and this model has been used to screen various compounds for potential therapeutic benefit. 15,19 However, exclusive reliance upon muscle force is an inadequate method of assessing the potential therapeutic benefit of various compounds. 9,41 There are few therapeutic options available to boys with DMD; however, corticosteroid treatment has resulted in improved function and strength.…”

mentioning

confidence: 99%

“…Creatine monohydrate (CrM) supplementation increases strength in mdx mice 15 and patients with muscular dystrophy. 25,47,52 It enhances survival and reduces intracellular calcium accumulation in cultured mdx myoblasts and in primary cell cultures from patients with DMD.…”

mentioning

confidence: 99%

See 3 more Smart Citations

Nutritional therapy improves function and complements corticosteroid intervention in mdx mice

et al. 2005

View full text Add to dashboard Cite

Corticosteroid therapy for Duchenne muscular dystrophy is effective but associated with long-term side effects. To determine the potential therapeutic benefit from four nutritional compounds (creatine monohydrate, conjugated linoleic acid, ␣-lipoic acid, and ␤-hydroxy-␤-methylbutyrate) alone, in combination, and with corticosteroids (prednisolone), we evaluated the effects on several variables in exercising mdx mice. Outcome measures included grip strength, rotarod performance, serum creatine kinase levels, muscle metabolites, internalized myonuclei, and retroperitoneal fat pad weight. In isolation, each nutritional treatment showed some benefit, with the combination therapy showing the most consistent benefits. Prednisolone and the combination therapy together provided the most consistent evidence of efficacy; increased peak grip strength (P Ͻ 0.05), decreased grip strength fatigue (P Ͻ 0.05), decreased number of internalized myonuclei (P Ͻ 0.01), and smaller retroperitoneal fat pad stores (P Ͻ 0.001). This study provided evidence for therapeutic benefit from a four-compound combination therapy alone, and in conjunction with corticosteroids in the mdx model of DMD.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Animalsmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 2 more Smart Citations

Nutritional therapy improves function and complements corticosteroid intervention in mdx mice

et al. 2005

View full text Add to dashboard Cite

show abstract

“…Different muscles have been analyzed in mdx mice after exercise (Weller et al 1990;Brussee et al 1997;Granchelli et al 2000;De Luca et al 2005), administration of drugs (Hodgetts et al 2006), engraftment of somatic cells (Hindi et al 2013;Boldrin et al 2015), and gene modification (Li et al 2009;Heydemann et al 2012). The results obtained and the skeletal muscles analyzed are different in these previous reports.…”

Section: Discussionmentioning

confidence: 99%

Degenerative and regenerative features of myofibers differ among skeletal muscles in a murine model of muscular dystrophy

Ikeda

Ichii

Otsuka-Kanazawa

et al. 2016

J Muscle Res Cell Motil

View full text Add to dashboard Cite

Skeletal muscle myofibers constantly undergo degeneration and regeneration. Histopathological features of 6 skeletal muscles (cranial tibial [CT], gastrocnemius, quadriceps femoris, triceps brachii [TB], lumbar longissimus muscles, and costal part of the diaphragm [CPD]) were compared using C57BL/10ScSn-Dmd mdx (mdx) mice, a model for muscular dystrophy versus control, C57BL/10 mice.Body weight and skeletal muscle mass were lower in mdx mice than the control at 4 weeks of age; these results were similar at 6-30 weeks. Additionally, muscular lesions were observed in all examined skeletal muscles in mdx mice after 4 weeks, but none were noted in the controls. Immunohistochemical staining revealed numerous paired box 7-positive satellite cells surrounding the embryonic myosin heavy chain-positive regenerating myofibers, while the number of the former and staining intensity of the latter decreased as myofiber regeneration progressed. Persistent muscular lesions were observed in skeletal muscles of mdx mice between 4-14 weeks of age, and normal myofibers decreased with age. Number of muscular lesions was lowest in CPD at all ages examined, while the ratio of normal myofibers was lowest in TB at 6 weeks. In CT, TB, and CPD, Iba1-positive macrophages, the main inflammatory cells in skeletal muscle lesions, showed a significant positive correlation with the appearance of regenerating myofibers. Additionally, B220-positive B-cells showed positive and negative correlation with regenerating and regenerated myofibers, respectively. Our data suggest that degenerative and regenerative features of myofibers differ among skeletal muscles and that inflammatory cells are strongly associated with regenerative features of myofibers in mdx mice.

show abstract

Zebrafish Assays for Identifying Potential Muscular Dystrophy Drug Candidates

Tang

Haldi

et al. 2011

Zebrafish

View full text Add to dashboard Cite

Pre-clinical screening of drugs using the mdx mouse

Cited by 144 publications

References 26 publications

Nutritional therapy improves function and complements corticosteroid intervention in mdx mice

Nutritional therapy improves function and complements corticosteroid intervention in mdx mice

Degenerative and regenerative features of myofibers differ among skeletal muscles in a murine model of muscular dystrophy

Zebrafish Assays for Identifying Potential Muscular Dystrophy Drug Candidates

Contact Info

Product

Resources

About