A modified diet does not ameliorate muscle pathology in a mouse model for Duchenne muscular dystrophy

Verhaart, Ingrid E.C.; Vijver, Davy van de; Meulen, Joke W. Boertje-van der; Putker, Kayleigh; Adamzek, Kevin; Aartsma-Rus, Annemieke; Putten, Maaike van

doi:10.1371/journal.pone.0215335

Cited by 3 publications

(4 citation statements)

References 53 publications

(55 reference statements)

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“…Therefore cardiac fibrosis found in D2-mdx at 25 weeks of age is most likely attributable to the DBA/2J background (Hakim et al, 2017). Also, calcification observed in the skeletal and heart muscles is uncommonly found in DMD patients, further emphasizing the limitations of using D2-mdx mice to understand the full systemic impact of DMD-targeting therapies (Hakim et al, 2017;Verhaart et al, 2019).…”

Section: Discussionmentioning

confidence: 99%

A novel mouse model of Duchenne muscular dystrophy carrying a multi-exonic Dmd deletion exhibits progressive muscular dystrophy and early-onset cardiomyopathy

Wong

Ahmed

Yang

et al. 2020

Disease Models &Amp; Mechanisms

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Duchenne muscular dystrophy (DMD) is a life-threatening neuromuscular disease caused by the lack of dystrophin, resulting in progressive muscle wasting and locomotor dysfunctions. By adulthood, almost all patients also develop cardiomyopathy, which is the primary cause of death in DMD. Although there has been extensive effort in creating animal models to study treatment strategies for DMD, most fail to recapitulate the complete skeletal and cardiac disease manifestations that are presented in affected patients. Here, we generated a mouse model mirroring a patient deletion mutation of exons 52-54 (Dmd Δ52-54). The Dmd Δ52-54 mutation led to the absence of dystrophin, resulting in progressive muscle deterioration with weakened muscle strength. Moreover, Dmd Δ52-54 mice present with early-onset hypertrophic cardiomyopathy, which is absent in current pre-clinical dystrophin-deficient mouse models. Therefore, Dmd Δ52-54 presents itself as an excellent pre-clinical model to evaluate the impact on skeletal and cardiac muscles for both mutation-dependent and -independent approaches.

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Section: Discussionmentioning

confidence: 99%

A novel mouse model of Duchenne muscular dystrophy carrying a multi-exonic Dmd deletion exhibits progressive muscular dystrophy and early-onset cardiomyopathy

Wong

Ahmed

Yang

et al. 2020

Disease Models &Amp; Mechanisms

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“…Otherwise, no effects of UA treatment in mdx mice were observed, even with higher administration doses; i.e. 0.02 % or around 25mg/kg of UA (Verhaart et al, 2019). However, using DBA/2Jmdx lineage, while we used a lineage with less intense dystrophic phenotype (C57BL/10-mdx).…”

Section: Discussionmentioning

confidence: 98%

Ursolic Acid Increases Strength in mdx Mice Model and may Decrease Fibrosis Deposition by TGF-ß Downregulation

Barreto¹,

Oliveira

Matias³

et al. 2022

Int. J. Morphol.

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Dystrophin disfunction results in sarcolemma destabilization, leading muscle cell damage by continuous degeneration cycles and limited regeneration. In muscle dystrophy, caused by dystrophin dysfunction, inflammation, necrosis and fibrosis are pathophysiological muscle function loss characteristics. As a genetic disease,this muscle dystrophy has no cure, however, advances in drug therapy using glucocorticoids can decrease the disease progression. Subsequently, alternative therapies were studied, such as ursolic acid (UA), that inhibits muscle atrophy and increases muscle mass and strength. Herein, we used 10 mg/kg daily supplementation in mdx mice for 4 weeks to evaluate serum creatine phosphokinase (CPK), muscle strength (Kondziela test), muscular organization (histology) and expression of fibrosis related genes (TGF-ß, TNF-α, mstn and ostn). UA supplementation increased muscle morphological organization, motor strength and decreased muscular TGF-ß expression. Altogether, the gene expression profile, histological organization and strength could suggest that UA treatment did not stop the fibrogenesis but decreased its progress.

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“…It has been reported that individuals of age 4-13, 14-18 and 19 years should consume 0.95, 0.85 and 0.80g/kg weight/day protein, respectively [11]. The effects of this amount on disease pathology in mouse models are shown to be uncertain [53,54]. Some studies report that high protein diets do not have any positive effect [18].…”

Section: Protein Requirementmentioning

confidence: 99%

“…Some studies report that high protein diets do not have any positive effect [18]. Limited findings on the positive effects of additional protein intake on the disease suggest that additional protein intake is not necessary in these patients [9,12,53].…”

Section: Protein Requirementmentioning

confidence: 99%

Nutritional Problems in Patients with Duchenne Muscular Dystrophy

Berberoğlu¹,

Tek

2021

BJSTR

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Duchenne muscular dystrophy (DMD) is an X-linked recessive hereditary disorder of muscles. DMD, which is a multisystemic disease, patients often experience gastrointestinal problems such as gastroesophageal reflux, constipation, dysphagia, delayed gastric motility, dental and jaw structure disorders and chewing disorders, in addition to musculoskeletal, pulmonary and heart problems. These problems, which increase with age, increase the risk of developing obesity and malnutrition and worsen the disease progression. This decreases the quality of life of patients and those who care for patients. There is no definite treatment for DMD disease. In addition to medical treatment, nutritional regulations ensure that patients receive enough daily energy, protein, various micronutrients and fluid, protect patients from obesity and malnutrition, and make dietary modifications in line with the presence of nutritional and gastrointestinal problems is important. Dietary modifications such as changing the texture of foods, dividing the foods into small pieces, choosing foods with high energy content or more liquid foods should be considered as needed. If necessary, gastrostomy or enteral feeding options should also be considered. Although there are data on the benefits of dietary supplements, information on the use of these products in individuals with DMD is limited. In this review, common nutritional problems in patients with DMD, dietary modifications related to the management of these problems and nutritional therapy will be evaluated.

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A modified diet does not ameliorate muscle pathology in a mouse model for Duchenne muscular dystrophy

Cited by 3 publications

References 53 publications

A novel mouse model of Duchenne muscular dystrophy carrying a multi-exonic Dmd deletion exhibits progressive muscular dystrophy and early-onset cardiomyopathy

A novel mouse model of Duchenne muscular dystrophy carrying a multi-exonic Dmd deletion exhibits progressive muscular dystrophy and early-onset cardiomyopathy

Ursolic Acid Increases Strength in mdx Mice Model and may Decrease Fibrosis Deposition by TGF-ß Downregulation

Nutritional Problems in Patients with Duchenne Muscular Dystrophy

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