Histopathological Evaluation of Skeletal Muscle with Specific Reference to Mouse Models of Muscular Dystrophy

Terry, Rebecca; Wells, Dominic J.

doi:10.1002/cpmo.19

Cited by 9 publications

(9 citation statements)

References 25 publications

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“…The DIA muscle was longitudinally divided into 2 halves. One half was frozen immediately in liquid nitrogen for RNA work while the other half was prepared as described in [ 39 ] for histological analyses. OCT-embedded frozen muscles were cryosectioned on an OTF5000 cryostat (Bright, Huntingdon, UK) at a 10 µm thickness for 10 serial levels through the muscle length, and the transverse sections were collected onto SuperFrost slides (VWR, Lutterworth, UK).…”

Section: Methodsmentioning

confidence: 99%

Long-Term Systemic Treatment of a Mouse Model Displaying Chronic FSHD-like Pathology with Antisense Therapeutics That Inhibit DUX4 Expression

et al. 2022

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Silencing the expression of the double homeobox 4 (DUX4) gene offers great potential for the treatment of facioscapulohumeral muscular dystrophy (FSHD). Several research groups have recently reported promising results using systemic antisense therapy in a transgenic small animal model of FSHD, the ACTA1-MCM/FLExDUX4 mouse model. However, the treatment was applied in non-DUX4-induced mice or shortly after DUX4 activation, which resulted in conditions that do not correctly represent the situation in a clinic. Here, we generated progressive FSHD-like pathology in ACTA1-MCM/FLExDUX4 mice and then treated the animals with vivoPMO-PACS4, an antisense compound that efficiently downregulates DUX4. To best mimic the translation of this treatment in clinical settings, the systemic antisense oligonucleotide administration was delayed to 3 weeks after the DUX4 activation so that the pathology was established at the time of the treatment. The chronic administration of vivoPMO-PACS4 for 8 weeks downregulated the DUX4 expression by 60%. Consequently, the treated mice showed an increase by 18% in body-wide muscle mass and 32% in muscle strength, and a reduction in both myofiber central nucleation and muscle fibrosis by up to 29% and 37%, respectively. Our results in a more suitable model of FSHD pathology confirm the efficacy of vivoPMO-PACS4 administration, and highlight the significant benefit provided by the long-term treatment of the disease.

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Section: Methodsmentioning

confidence: 99%

Long-Term Systemic Treatment of a Mouse Model Displaying Chronic FSHD-like Pathology with Antisense Therapeutics That Inhibit DUX4 Expression

et al. 2022

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“…Gastrocnemius and soleus were extracted and iced in isopentane cooled in liquid nitrogen as described previously 40 . Sections 8 µm of transversal red gastrocnemius and soleus muscles were cut in cryostat.…”

Section: Methodsmentioning

confidence: 99%

“…Gastrocnemius and soleus were extracted and iced in isopentane cooled in liquid nitrogen as described previously. 40 Sections 8 µm of transversal red gastrocnemius and soleus muscles were cut in cryostat. For succinate dehydrogenase (SDH) stain, sections were incubated at 37°C in a wet chamber for 45 min with a solution with phosphate buffer 0.2 M (pH 7,4), succinic acid 0.2 M (Sigma Chemical Co.), and nitroblue tetrazolium 2.4 mM of (NBT, Sigma Chemical Co.).…”

Section: Sdh and Cox Stain In Muscle Biopsiesmentioning

confidence: 99%

Skeletal muscle type‐specific mitochondrial adaptation to high‐fat diet relies on differential autophagy modulation

et al. 2021

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In obesity, skeletal muscle mitochondrial activity changes to cope with increased nutrient availability. Autophagy has been proposed as an essential mechanism involved in the regulation of mitochondrial metabolism. Still, the contribution of autophagy to mitochondrial adaptations in skeletal muscle during obesity is unknown. Here, we show that in response to high-fat diet (HFD) feeding, distinct skeletal muscles in mice exhibit differentially regulated autophagy that may modulate mitochondrial activity. We observed that after 4 and 40 weeks of high-fat diet feeding, OXPHOS subunits and mitochondrial DNA content increased in the oxidative soleus muscle. However, in gastrocnemius muscle, which has a mixed fiber-type composition, the mitochondrial mass increased only after 40 weeks of HFD feeding. Interestingly, fatty acid-supported mitochondrial respiration was enhanced in gastrocnemius, but not in soleus muscle after a 4-week HFD feeding. This increased metabolic profile in gastrocnemius was paralleled by preserving autophagy flux, while autophagy flux in soleus was reduced. To determine the

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“…The fact that skeletal muscle tissue is susceptible to only few pathological changes is not surprising. The basic histopathological patterns recognizable under optical microscopy are few: normal trophism, hypotrophy, hypertrophy, dystrophy, and myositis [10,11,12]. "Myopathy" is a non-specific term meaning intrinsic muscle disease.…”

Section: Discussionmentioning

confidence: 99%

Atrophy and Muscular Fibrosis of Unknown Etiology during the Raising of Xiphophorus maculattus on an Ornamental Fish Farm

Romano

Medeiros

Pedrosa

2020

AJRiZ

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During the last two decades, the implementation of histochemical, immunohistochemical, electron microscopy, and recently developed molecular techniques has greatly contributed to our knowledge of skeletal muscle, both normal and sick. This article reports the presence of muscular atrophy and fibrosis in Xiphophorus maculatus from an ornamental fish farm. We do not know the origin of this muscular pathology and the purpose of this work is to summarize some of the findings with optical microscopy and electron microscopy shared by all. Although we cannot establish the etiology of this atrophy, we will try to correlate the ultrastructural alterations with the muscular histopathology. Muscular atrophy is a pathology characterized by loss of normal muscle mass. It is caused by a decrease in the total number of muscle cells or by a substantial reduction in the substance of individual muscle cells. It is likely that the cases reported here represent a pathology involving causes concurrent with nutritional problems and disorders of muscle innervation. Therefore, future studies should investigate further about the potential of neurodegenerative disorders. Several experimental models can use muscular atrophy and are suitable for investigations of the underlying mechanisms of this pathology.

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Histopathological Evaluation of Skeletal Muscle with Specific Reference to Mouse Models of Muscular Dystrophy

Cited by 9 publications

References 25 publications

Long-Term Systemic Treatment of a Mouse Model Displaying Chronic FSHD-like Pathology with Antisense Therapeutics That Inhibit DUX4 Expression

Long-Term Systemic Treatment of a Mouse Model Displaying Chronic FSHD-like Pathology with Antisense Therapeutics That Inhibit DUX4 Expression

Skeletal muscle type‐specific mitochondrial adaptation to high‐fat diet relies on differential autophagy modulation

Atrophy and Muscular Fibrosis of Unknown Etiology during the Raising of Xiphophorus maculattus on an Ornamental Fish Farm

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