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J. Funct. Morphol. Kinesiol. 2017, 2(4), 44; https://doi.org/10.3390/jfmk2040044

Insights into the Pathogenic Secondary Symptoms Caused by the Primary Loss of Dystrophin

1
DAHFMO-Unit of Histology and Medical Embryology, Laboratory Affiliated to Istituto Pasteur Italia—Fondazione Cenci Bolognetti, Sapienza University of Rome, 00161 Rome, Italy
2
Center for Life Nano Science@Sapienza, Istituto Italiano di Tecnologia, 00161 Rome, Italy
*
Author to whom correspondence should be addressed.
Received: 30 October 2017 / Revised: 24 November 2017 / Accepted: 30 November 2017 / Published: 2 December 2017
(This article belongs to the Special Issue Muscular Dystrophy)
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Abstract

Duchenne muscular dystrophy (DMD) is an X-linked genetic disease in which the dystrophin gene is mutated, resulting in dysfunctional dystrophin protein. Without dystrophin, the dystrophin-glycoprotein complex (DGC) is unstable, leading to an increase in muscle damage. Moreover, the imbalance between muscle damage and repair leads to a chronic inflammatory response and an increase in the amount of fibrosis over time. The absence of dystrophin at the sarcolemma also delocalizes and downregulates nitric oxide synthase (nNOS) and alters enzymatic antioxidant responses, leading to an increase in oxidative stress. In this review, we analyze the pathogenic role of both inflammation and oxidative stress in muscular dystrophy. View Full-Text
Keywords: Duchenne muscular dystrophy; inflammation; oxidative stress; therapeutic approaches; muscle wasting Duchenne muscular dystrophy; inflammation; oxidative stress; therapeutic approaches; muscle wasting
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Forcina, L.; Pelosi, L.; Miano, C.; Musarò, A. Insights into the Pathogenic Secondary Symptoms Caused by the Primary Loss of Dystrophin. J. Funct. Morphol. Kinesiol. 2017, 2, 44.

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