Progression of kyphosis in mdx mice

Article


Laws, Nicola and Hoey, Andrew. 2004. "Progression of kyphosis in mdx mice." Journal of Applied Physiology. 97 (5), pp. 1970-1977. https://doi.org/10.1152/japplphysiol.01357.2003
Article Title

Progression of kyphosis in mdx mice

ERA Journal ID3161
Article CategoryArticle
AuthorsLaws, Nicola (Author) and Hoey, Andrew (Author)
Journal TitleJournal of Applied Physiology
Journal Citation97 (5), pp. 1970-1977
Number of Pages8
Year2004
PublisherAmerican Physiological Society
Place of PublicationUnited States
ISSN1522-1601
8750-7587
Digital Object Identifier (DOI)https://doi.org/10.1152/japplphysiol.01357.2003
Web Address (URL)https://journals.physiology.org/doi/full/10.1152/japplphysiol.01357.2003
Abstract

Spinal deformity in the form of kyphosis or kyphoscoliosis occurs in most patients with Duchenne muscular dystrophy (DMD), a fatal X-linked disorder caused by an absence of the subsarcolemmal protein dystrophin. Mdx mice, which also lack dystrophin, show thoracolumbar kyphosis that progresses with age. We hypothesize that paraspinal and respiratory muscle weakness and fibrosis are associated with the progression of spinal deformity in this mouse model, and similar to DMD patients there is evidence of altered thoracic conformation and area. We measured kyphosis in mdx and age-matched control mice by monthly radiographs and the application of a novel radiographic index, the kyphotic index, similar to that used in boys with DMD. Kyphotic index became significantly less in mdx at 9 mo of age (3.58 ± 0.12 compared with 4.27 ± 0.04 in the control strain; P ≤ 0.01), indicating more severe kyphosis, and remained less from 10 to 17 mo of age. Thoracic area in 17-mo-old mdx was reduced by 14% compared with control mice (P ≤ 0.05). Peak tetanic tension was significantly lower in mdx and fell 47% in old mdx latissimus dorsi muscles, 44% in intercostal strips, and 73% in diaphragm strips (P ≤ 0.05). Fibrosis of these muscles and the longissimus dorsi, measured by hydroxyproline analysis and histological grading of picrosirius red-stained sections, was greater in mdx (P < 0.05). We conclude that kyphotic index is a useful measure in mdx and other kyphotic mouse strains, and assessment of paralumbar and accessory respiratory muscles enhance understanding of spinal deformity in muscular dystrophy.

Keywordsespiratory muscles; latissimus dorsi; intercostal muscles; kyphotic index; hydroxyproline
ANZSRC Field of Research 2020310999. Zoology not elsewhere classified
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Byline AffiliationsDepartment of Biological Physical Sciences
Department of Biological and Physical Sciences
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