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Microsc Res Tech. 2014 Nov;77(11):857-61. doi: 10.1002/jemt.22408. Epub 2014 Aug 1.

Morphological and ultrastructural evaluation of the golden retriever muscular dystrophy trachea, lungs, and diaphragm muscle.

Author information

1
Department of Surgery, Faculdade de Medicina Veterinária e Zootecnia, Universidade de São Paulo, FMVZ/USP, Prof. Dr. Orlando Marques de Paiva, n. 87, Cidade Universitária, 05508-000, São Paulo, SP, Brazil.

Abstract

Duchenne muscular dystrophy (DMD) is a genetic disease, characterized by atrophy and muscle weakness. The respiratory failure is a common cause of early death in patients with DMD. Golden retriever muscular dystrophy (GRMD) is a canine model which has been extensively used for many advances in therapeutics applications. As the patients with DMD, the GRMD frequently died from cardiac and respiratory failure. Observing the respiratory failure in DMD is one of the major causes of mortality we aimed to describe the morphological and ultrastructural data of trachea, lungs (conductive and respiratory portion of the system), and diaphragm muscle using histological and ultrastructural analysis. The diaphragm muscle showed discontinuous fibers architecture, with different diameter; a robust perimysium inflammatory infiltrate and some muscle cells displayed central nuclei. GRMD trachea and lungs presented collagen fibers and in addition, the GRMD lungs showed higher of levels collagen fibers that could limit the alveolar ducts and alveoli distension. Therefore, the most features observed were the collagen areas and fibrosis. We suggested in this study that the collagen remodeling in the trachea, lungs, and diaphragm muscle may increase fibrosis and affect the trachea, lungs, and diaphragm muscle function that can be a major cause of respiratory failure that occur in patients with DMD.

KEYWORDS:

Canine model; Duchenne; genetic disease; respiratory failure

PMID:
25081087
DOI:
10.1002/jemt.22408
[Indexed for MEDLINE]

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