Motor dysfunction of the gut in Duchenne muscular dystrophy: A review

被引:0
|
作者
Subhan, Fazal [1 ]
Zizzo, Maria Grazia [1 ,2 ]
Serio, Rosa [1 ]
机构
[1] Univ Palermo, Dept Biol Chem & Pharmaceut Sci & Technol STEBICEF, Viale Sci, I-90128 Palermo, Italy
[2] Univ Palermo, ATeN Adv Technol Network Ctr, Viale Sci, Palermo, Italy
来源
NEUROGASTROENTEROLOGY AND MOTILITY | 2024年 / 36卷 / 08期
关键词
Duchenne muscular dystrophy (DMD); dystrophin; gastrointestinal tract; mdx mice; smooth muscle; NITRIC-OXIDE SYNTHASE; ACUTE GASTRIC DILATATION; MDX MOUSE MODEL; INTERSTITIAL-CELLS; SMOOTH-MUSCLE; INTESTINAL PSEUDOOBSTRUCTION; GASTROINTESTINAL-TRACT; MECHANICAL-ACTIVITY; MICE; CAJAL;
D O I
10.1111/nmo.14804
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
BackgroundDuchenne's muscular dystrophy (DMD) is a severe type of hereditary, neuromuscular disorder caused by a mutation in the dystrophin gene resulting in the absence or production of truncated dystrophin protein. Conventionally, clinical descriptions of the disorder focus principally on striated muscle defects; however, DMD manifestations involving gastrointestinal (GI) smooth muscle have been reported, even if not rigorously studied. PurposeThe objective of the present review is to offer a comprehensive perspective on the existing knowledge concerning GI manifestations in DMD, focusing the attention on evidence in DMD patients and mdx mice. This includes an assessment of symptomatology, etiological pathways, and potential corrective approaches. This paper could provide helpful information about DMD gastrointestinal implications that could serve as a valuable orientation for prospective research endeavors in this field. This manuscript emphasizes the effectiveness of mdx mice, a DMD animal model, in unraveling mechanistic insights and exploring the pathological alterations in the GI tract. The gastrointestinal consequences evident in patients with DMD and the mdx mice models are a significant area of focus for researchers. The exploration of this area in depth could facilitate the development of more efficient therapeutic approaches and improve the well-being of individuals impacted by the condition.
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页数:13
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