在胶原蛋白VI相关肌病的Col6a1小鼠模型中，沙丁胺醇的新用途改善了神经肌肉接头缺陷和肌肉萎缩。 - Suppr

Salbutamol repurposing ameliorates neuromuscular junction defects and muscle atrophy in Col6a1 mouse model of collagen VI-related myopathies.

作者信息

Calabrò Sonia, Nogara Leonardo, Jian Yongzhi, Valentin Manuel, Bizzotto Dario, Braghetta Paola, Russo Loris, Gambarotto Lisa, Blaauw Bert, Hashemolhosseini Said, Bonaldo Paolo, Cescon Matilde

机构信息

Department of Molecular Medicine, University of Padova, Padova, Italy.

Department of Biology, University of Padova, Padova, Italy.

出版信息

Clin Transl Med. 2024 Jul;14(7):e1688. doi: 10.1002/ctm2.1688.

DOI:10.1002/ctm2.1688

PMID:38984773

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11234414/

Abstract

摘要

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6d59/11234414/56b05fe35cf4/CTM2-14-e1688-g002.jpg

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Salbutamol repurposing ameliorates neuromuscular junction defects and muscle atrophy in Col6a1 mouse model of collagen VI-related myopathies.在胶原蛋白VI相关肌病的Col6a1小鼠模型中，沙丁胺醇的新用途改善了神经肌肉接头缺陷和肌肉萎缩。

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Hum Mol Genet. 2019 Jul 15;28(14):2339-2351. doi: 10.1093/hmg/ddz059.

Collagen VI is required for the structural and functional integrity of the neuromuscular junction.胶原 VI 对于神经肌肉接头的结构和功能完整性是必需的。

Acta Neuropathol. 2018 Sep;136(3):483-499. doi: 10.1007/s00401-018-1860-9. Epub 2018 May 11.

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Autophagy. 2015;11(12):2142-52. doi: 10.1080/15548627.2015.1108508.

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Neurobiol Dis. 2016 Jan;85:11-24. doi: 10.1016/j.nbd.2015.10.006. Epub 2015 Oct 14.

Salbutamol benefits children with congenital myasthenic syndrome due to DOK7 mutations.沙丁胺醇有益于 DOK7 突变导致的先天性肌无力综合征患儿。

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Role of beta-adrenoceptor signaling in skeletal muscle: implications for muscle wasting and disease.β-肾上腺素能受体信号在骨骼肌中的作用：对肌肉萎缩和疾病的影响。

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Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies.环孢素A可纠正胶原蛋白VI型肌病患者的线粒体功能障碍和肌肉细胞凋亡。

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Collagen VI deficiency induces early onset myopathy in the mouse: an animal model for Bethlem myopathy.胶原蛋白VI缺乏在小鼠中诱发早发性肌病：一种贝思伦肌病的动物模型。

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Salbutamol repurposing ameliorates neuromuscular junction defects and muscle atrophy in Col6a1 mouse model of collagen VI-related myopathies.

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