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营养不良性肌萎缩症对 mdx 小鼠部分内在性喉肌的影响。

Effect of dystrophin deficiency on selected intrinsic laryngeal muscles of the mdx mouse.

机构信息

Department of Communication Disorders, Marshall University, 1 John Marshall Drive, Huntington, WV 25755, USA.

出版信息

J Speech Lang Hear Res. 2010 Jun;53(3):633-47. doi: 10.1044/1092-4388(2009/09-0010). Epub 2009 Dec 14.

Abstract

BACKGROUND

Intrinsic laryngeal muscles (ILM) show biological differences from the broader class of skeletal muscles. Yet most research regarding ILM specialization has been completed on a few muscles, most notably the thyroarytenoid and posterior cricoarytenoid. Little information exists regarding the biology of other ILM. Early evidence suggests that the interarytenoid (IA) and cricothyroid (CT) may be more similar to classic skeletal muscle than their associated laryngeal muscles. Knowledge of the IA and CT's similarity or dissimilarity to typical skeletal muscle may hold implications for the treatment of dysphonia.

PURPOSE

The purpose of this study was to further define IA and CT biology by examining their response to the biological challenge of dystrophin deficiency.

METHOD

Control and dystrophin-deficient superior cricoarytenoid (SCA; mouse counterpart of IA) and CT muscles were examined for fiber morphology, sarcolemmal integrity, and immunohistochemical detection of dystrophin.

RESULTS

Despite the absence of dystrophin, experimental muscles did not show disease markers.

CONCLUSIONS

The SCA and the CT appear spared in dystrophin-deficient mouse models. These laryngeal muscles possess specializations that separate them from typical skeletal muscle. Considered in light of previous research, the CT and IA may represent transitional form of muscle, evidencing properties of typical and specialized skeletal muscle.

摘要

背景

固有喉肌(ILM)与更广泛的骨骼肌在生物学上存在差异。然而,关于 ILM 特化的大多数研究都是在少数肌肉上完成的,尤其是甲状腺肌和后环杓肌。关于其他 ILM 的生物学信息很少。早期证据表明,杓间肌(IA)和环甲肌(CT)可能与经典骨骼肌更相似,而不是它们所关联的喉肌。了解 IA 和 CT 与典型骨骼肌的相似性或差异性可能对治疗声音障碍具有重要意义。

目的

本研究的目的是通过检查其对营养不良蛋白缺乏这一生物学挑战的反应,进一步定义 IA 和 CT 的生物学特性。

方法

对对照和营养不良蛋白缺陷型杓状软骨上肌(SCA;IA 的小鼠对应物)和 CT 肌肉进行纤维形态、肌膜完整性以及营养不良蛋白免疫组织化学检测。

结果

尽管缺乏营养不良蛋白,但实验性肌肉并未显示出疾病标志物。

结论

在营养不良蛋白缺陷型小鼠模型中,SCA 和 CT 似乎未受影响。这些喉肌具有使其与典型骨骼肌分离的特化功能。考虑到之前的研究,CT 和 IA 可能代表了肌肉的过渡形式,表现出典型和特化骨骼肌的特性。

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