Fibrogenesis in LAMA2-Related Muscular Dystrophy Is a Central Tenet of Disease Etiology

Accorsi, Anthony; Cramer, Megan L.; Girgenrath, Mahasweta

doi:10.3389/fnmol.2020.00003

Cited by 24 publications

(18 citation statements)

References 90 publications

(103 reference statements)

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“…Another enzyme useful for diagnosis and monitoring of muscular disorders is lactate dehydrogenase (LDH) which converts pyruvate to lactate. Its drawback is that many tissues other than muscles have high concentrations of LDH which is released upon cell membrane dysfunction (13).…”

Section: Introductionmentioning

confidence: 99%

Ascorbic Acid Greatly Decreases Creatine Kinase Levels in An Animal Model of Statin/Fibrate-Induced Myopathy

Askarian

Zabihi

Hekmatimoghaddam

et al. 2021

Preprint

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BackgroundDrug-induced myopathy is one of the frequent forms of muscle disease, and drugs used for hyperlipidemia, especially the statins are a common culprit, and particularly when combined with a fibrate. Clinicians usually measure plasma levels of three enzymes, creatine kinase (CK), aldolase and lactate dehydrogenase (LDH) for diagnosis of myopathy and determination of its severity. Physical exercise can aggravate statin-associated muscular disease. The question is whether antioxidants like ascorbic acid (vit. C) can prevent such myopathy.MethodsIn this experiment a combination of oral atorvastatin (ATV, 80 mg/kg/day, orally) and gemfibrozil (GMF, 1000 mg/kg/day, orally) was used for ten days plus exercise in days 8, 9 and 10 to induce myopathy in rats. To add physical exercise, the forced swimming test was applied in the last three days. Ascorbic acid (50 mg/kg/day, orally) was added to ATV/GMF plus exercise regimen throughout the 10 days in the treatment group. The mean blood levels of CK, aldolase and LDH were measured in addition to swimming tolerance times. ResultsThere was a significantly lower swimming tolerance time (P < 0.05) and higher CK levels (P < 0.01) in rats receiving ATV/GMF/Vit.C plus exercise compared with rats not taking Vit.C. LDH and aldolase didn’t decrease significantly.ConclusionsA protective role of vit.C against drug-induced myopathy is suggested by the findings of this study.

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Section: Introductionmentioning

confidence: 99%

Ascorbic Acid Greatly Decreases Creatine Kinase Levels in An Animal Model of Statin/Fibrate-Induced Myopathy

Askarian

Zabihi

Hekmatimoghaddam

et al. 2021

Preprint

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“…al, 2019). Essa inflamação crônica em indivíduos com MDC1A é notada desde poucos meses do nascimento; a fibrose, no entanto, parece ser uma resposta mais tardia ao processo crônico inflamatório (Accorsi et. al, 2020).…”

Section: Introductionunclassified

Efeitos do treinamento muscular inspiratório na performance muscular inspiratória de um paciente com distrofia muscular congênita com deficiência de merosina: um relato de caso

Souza

Formiga

Vania

et al. 2021

RSD

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A distrofia muscular congênita com deficiência da merosina (MDC1A) é caracterizada por um comprometimento da fibra muscular pela deficiência parcial ou completa da laminina α2 (merosina) uma proteína extracelular responsável pela manutenção da estrutura celular. A alteração da biomecânica nas estruturas envolvidas com o ato respiratório pode desencadear fraqueza muscular respiratória e baixa resistência à fadiga, evidenciando quadros de desconforto respiratório e outros agravos da função pulmonar. Em resposta a isso, os efeitos do Treinamento Muscular Respiratório (TMR) têm sido explanados ao longo dos anos na literatura. Apesar de conhecidos os benefícios do TMR, não há um consenso definido em relação a protocolos e tipo de TMR a ser utilizado em pacientes com afecções neuromusculares, especialmente em crianças. Dado o exposto, o presente estudo tem por objetivo determinar os efeitos de um protocolo de TMR sobre a força muscular respiratória e qualidade de vida de paciente com Distrofia Muscular Congênita (DMC) com deficiência de merosina (DM).

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“…Another valuable enzyme for diagnosing and monitoring muscular disorders is lactate dehydrogenase (LDH), which converts pyruvate to lactate. Its drawback is that many tissues other than muscles have high concentrations of LDH, which are released upon cell membrane dysfunction [ 13 ].…”

Section: Introductionmentioning

confidence: 99%

Ascorbic Acid Significantly Decreases Creatine Kinase Plasma Levels in an Animal Model of Statin/Fibrate-Induced Myopathy

Zabihi

Askarian

Hekmatimoghaddam

et al. 2021

Advances in Pharmacological and Pharmaceutical Sciences

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Background. Myopathy is one of the side effects of lipid-lowering drugs, especially statins and particularly when combined with a fibrate. To diagnose myopathy and determine its severity, the plasma levels of three enzymes, creatine kinase (CK), aldolase, and lactate dehydrogenase (LDH), are routinely measured. Physical exercise can aggravate the statin-associated muscular disease. The question is whether antioxidants like ascorbic acid (Vit. C) can prevent such myopathy. Methods. In this experiment, a combination of atorvastatin (ATV, 80 mg/kg/day) and gemfibrozil (GMF, 1000 mg/kg/day) orally for 10 days as well as exercise as forced swimming on days 8, 9, and 10 were used to induce myopathy. Ascorbic acid (50 mg/kg/day, orally) was added to ATV/GMF plus exercise regimen throughout the 10 days in the treatment group. Mean blood levels of CK, aldolase, and LDH were measured in addition to swimming tolerance times. Results. There was a significantly higher swimming tolerance time P < 0.05 and lower CK levels P < 0.01 in rats receiving ATV/GMF/Vit. C plus exercise compared with rats not taking Vit. C. LDH and aldolase did not decrease significantly. Conclusion. The results of this study showed that Vit. C can be effective in preventing myopathy caused by fat-lowering drugs.

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Fibrogenesis in LAMA2-Related Muscular Dystrophy Is a Central Tenet of Disease Etiology

Cited by 24 publications

References 90 publications

Ascorbic Acid Greatly Decreases Creatine Kinase Levels in An Animal Model of Statin/Fibrate-Induced Myopathy

Ascorbic Acid Greatly Decreases Creatine Kinase Levels in An Animal Model of Statin/Fibrate-Induced Myopathy

Efeitos do treinamento muscular inspiratório na performance muscular inspiratória de um paciente com distrofia muscular congênita com deficiência de merosina: um relato de caso

Ascorbic Acid Significantly Decreases Creatine Kinase Plasma Levels in an Animal Model of Statin/Fibrate-Induced Myopathy

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