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Total 34 results found since Jan 2013.

G.P.41: Canine X-linked myotubular myopathy: A dose-finding study of systemic AAV8-MTM1 effects on muscle strength, gait, respiration, neuromuscular function and survival
Mutations in the myotubularin gene (MTM1) result in X-linked myotubular myopathy (XLMTM), a fatal pediatric disease of skeletal muscle characterized by small centrally nucleated myofibers containing abnormal mitochondrial accumulations. Patients typically present with severe hypotonia and respiratory failure. Previous local studies in Mtm1-mutant mice demonstrated potential efficacy of gene therapy to treat the disease. We recently reported that administration of adeno-associated virus serotype 8 (AAV8) vector expressing myotubularin under the muscle-specific desmin promoter, delivered systemically in myotubularin deficien...
Source: Neuromuscular Disorders - September 4, 2014 Category: Neurology Authors: D.L. Mack, M. Goddard, J.M. Snyder, J. Doering, M.W. Lawlor, P. Moullier, M. O’Callaghan, A.H. Beggs, F. Mavilio, K. Poulard, V. Latournerie, A. Buj-Bello, M. Childers Source Type: research

Peripheral vein injection of AAV8-MTM1 leads to long-term survival and correction of severe muscle pathology in a canine model of X-linked myotubular myopathy: Results from a dose escalation study
Mutations in the myotubularin gene (MTM1) result in X-linked myotubular myopathy (XLMTM), a fatal pediatric disease of the skeletal muscle characterized by small centrally nucleated myofibers containing abnormal mitochondrial accumulations. Patients typically present with severe hypotonia and respiratory failure. Previous studies in Mtm1 mutant mice demonstrated efficacy of gene therapy to treat the disease. We also previously reported that administration of an adeno-associated virus serotype 8 (AAV8) vector expressing myotubularin under the muscle-specific desmin promoter via high pressure regional hind limb perfusion pro...
Source: Neuromuscular Disorders - September 11, 2015 Category: Neurology Authors: D. Mack, K. Poulard, M. Goddard, J. Snyder, R. Grange, J. Doering, J. Strande, V. Latournerie, P. Veron, L. Yang, L. Buscara, C. Le Bec, S. Martin, M. O'Callaghan, F. Mingozzi, A. Beggs, M. Lawlor, F. Mavilio, M. Childers, A. Buj-Bello Source Type: research

G.p.41
Mutations in the myotubularin gene (MTM1) result in X-linked myotubular myopathy (XLMTM), a fatal pediatric disease of skeletal muscle characterized by small centrally nucleated myofibers containing abnormal mitochondrial accumulations. Patients typically present with severe hypotonia and respiratory failure. Previous local studies in Mtm1-mutant mice demonstrated potential efficacy of gene therapy to treat the disease. We recently reported that administration of adeno-associated virus serotype 8 (AAV8) vector expressing myotubularin under the muscle-specific desmin promoter, delivered systemically in myotubularin deficien...
Source: Neuromuscular Disorders - October 1, 2014 Category: Neurology Authors: D.L. Mack, M. Goddard, J.M. Snyder, J. Doering, M.W. Lawlor, P. Moullier, M. O’Callaghan, A.H. Beggs, F. Mavilio, K. Poulard, V. Latournerie, A. Buj-Bello, Source Type: research

Postanesthetic Neuropathy/Myopathy in the Nondependent Forelimb in 4 Horses
Abstract: Postanesthetic lameness in the form of myopathy or neuropathy is a well-known complication associated with equine general anesthesia and is most often reported in the dependent limb of horses treated in lateral recumbency. The aim of this case series was to report clinical data of 4 horses treated under general anesthesia in lateral recumbency and that developed postanesthetic neuropathy/myopathy in the nondependent forelimb. With anti-inflammatory and analgesic medical treatment, locomotor problems associated with neuropathy/myopathy resolved after 12–24 hours in all cases. The pathogenesis remains unclear. A ...
Source: Journal of Equine Veterinary Science - April 22, 2013 Category: Veterinary Research Authors: Maarten Oosterlinck, Stijn Schauvliege, Ann Martens, Frederik Pille Tags: Original Research Source Type: research

A BAG3 Coding Variant in Mice Determines Susceptibility to Ischemic Limb Muscle Myopathy by Directing Autophagy.
Conclusions -Taken together, our data demonstrate that genetic variation in BAG3 plays an important role in the prevention of ischemic tissue necrosis. These results highlight a pathway that preserves tissue survival and muscle function in the setting of ischemia. PMID: 28442482 [PubMed - as supplied by publisher]
Source: Circulation - April 25, 2017 Category: Cardiology Authors: McClung JM, McCord TJ, Ryan TE, Schmidt CA, Green TD, Southerland KW, Reinardy JL, Mueller SB, Venkatraman TN, Lascola CD, Keum S, Marchuk DA, Spangenburg EE, Dokun AO, Annex BH, Kontos CD Tags: Circulation Source Type: research

Pulmonary arterial hypertension-related myopathy: An overview of current data and future perspectives
Conclusions Skeletal muscles of PAH patients show a wide spectrum of cellular abnormalities that finally culminate in muscle atrophy and reduced contractility. Exercise training improves muscle function and bears a positive impact on clinical outcomes of PAH patients.
Source: Nutrition, Metabolism and Cardiovascular Diseases - November 6, 2014 Category: Nutrition Source Type: research

Mechanism of Hydrogen Sulfide Preconditioning-Associated Protection Against Ischemia-Reperfusion Injury Differs in Diabetic Heart That Develops Myopathy.
Abstract Hydrogen sulfide (H2S) is reported to be effective in the management of the myocardial ischemia-reperfusion (I/R) injury via PI3K/GSK3β pathway in normal rats. However, its efficacy against I/R in the presence of diabetic cardiomyopathy is relatively obscure. Thus, the present work aimed to find out H2S-mediated cardioprotection against I/R in diabetic cardiomyopathy and to evaluate its mode of action using Langendorff isolated heart perfusion system. The present work includes three groups of rat, viz. (i) normal, (ii) diabetes mellitus (DM: streptozotocin: 35 mg/kg; normal diet), and (iii) diabetes +...
Source: Cardiovascular Toxicology - July 16, 2019 Category: Cardiology Authors: Ansari M, Kurian GA Tags: Cardiovasc Toxicol Source Type: research

Activation of the Aryl Hydrocarbon Receptor in Muscle Exacerbates Ischemic Pathology in Chronic Kidney Disease
CONCLUSIONS: These findings establish AHR activation in muscle as a pivotal regulator of the ischemic limb pathology in CKD. Further, the totality of the results provides support for testing of clinical interventions that diminish AHR signaling in these conditions.PMID:37325935 | DOI:10.1161/CIRCRESAHA.123.322875
Source: Circulation Research - June 16, 2023 Category: Cardiology Authors: Nicholas Balestrieri Victoria Palzkill Caroline Pass Jianna Tan Zachary R Salyers Chatick Moparthy Ania Murillo Kyoungrae Kim Trace Thome Qingping Yang Kerri A O'Malley Scott A Berceli Feng Yue Salvatore T Scali Leonardo F Ferreira Terence E Ryan Source Type: research

Detection of Presymptomatically Latent Lesions Developing into Stroke-Like Episodes by Arterial Spin-Labeling Perfusion MRI in MELAS Patients (P03.162)
CONCLUSIONS: These findings suggest that regional ASL hyperperfusion in the presymptomatic phase precedes the appearance of upcoming stroke-like lesions, and that presymptomatic SEs had already occurred several months before the onset of clinical symptoms. ASL imaging has great potential for detecting presymptomatic stroke-like lesions and predicting the emergence of SEs.Supported by: In part by Grants-in-Aid for the Scientific Research on Innovative Areas from the Ministry of Education, Culture, Sports, Science and Technology of Japan and Young Scientists (B) from the Japan Society for the Promotion of Science, and the Re...
Source: Neurology - February 14, 2013 Category: Neurology Authors: Ikawa, M., Kimura, H., Muramatsu, T., Matsunaga, A., Tsujikawa, T., Yamamoto, T., Kosaka, N., Kinoshita, K., Yamamura, O., Hamano, T., Nakamoto, Y., Yoneda, M. Tags: P03 Cerebrovascular Disease III Source Type: research

Mitochondria: Role of citrulline and arginine supplementation in MELAS syndrome.
Abstract Mitochondria are found in all nucleated human cells and generate most of the cellular energy. Mitochondrial disorders result from dysfunctional mitochondria that are unable to generate sufficient ATP to meet the energy needs of various organs. Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a frequent maternally inherited mitochondrial disorder. There is growing evidence that nitric oxide (NO) deficiency occurs in MELAS syndrome and results in impaired blood perfusion that contributes significantly to several complications including stroke-like episodes, myop...
Source: The International Journal of Biochemistry and Cell Biology - January 8, 2014 Category: Biochemistry Authors: El-Hattab AW, Emrick LT, Chanprasert S, Craigen WJ, Scaglia F Tags: Int J Biochem Cell Biol Source Type: research