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Serum inflammatory cytokines as disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy

Piercy, Richard; Riddell, Dominique; Hildyard, John; Harron, Rachel; Wells, Dominic


Richard Piercy

Dominique Riddell

John Hildyard

Rachel Harron

Dominic Wells


Duchenne muscular dystrophy (DMD) is a fatal muscle-wasting disease, caused by mutations in the dystrophin gene, characterised by cycles of muscle degeneration, inflammation and regeneration. The DE50-MD dog is a canine model of DMD that closely mimics the human DMD phenotype. We quantified a panel of proteins associated with inflammation in the serum of DE50-MD dogs in order to identify biomarkers for future pre-clinical trials. Serum protein concentrations were quantified by Luminex assay in samples from wild-type (WT) and DE50-MD dogs aged between 3 and 18 months. Significantly higher concentrations of C-C motif chemokine ligand 2 (CCL2), granulocyte-macrophage colony-stimulating factor, keratinocyte chemotactic-like, TNF-, and interleukins (IL)-2, IL6, IL7, IL8, IL10, IL15 and IL18, were detected in DE50-MD compared to WT serum. Of these, CCL2 best differentiated the 2 genotypes. Relative quantity of CCL2 mRNA was greater in DE50-MD vastus lateralis muscle than WT dogs and immunohistochemistry showed co-localisation of CCL2 with leucocyte marker CD18 in areas of muscle with signs of recent degeneration in DE50-MD samples. In conclusion, the serum cytokine profile suggests that inflammation is a feature of the DE50-MD phenotype. Quantification of serum CCL2 in particular is a useful non-invasive biomarker of DE50-MD pathology.


Piercy, R., Riddell, D., Hildyard, J., Harron, R., & Wells, D. (2022). Serum inflammatory cytokines as disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy. Neuromuscular Disorders,

Journal Article Type Article
Acceptance Date Dec 7, 2022
Publication Date Dec 9, 2022
Deposit Date Jan 12, 2021
Publicly Available Date Mar 24, 2023
Print ISSN 0960-8966
Publisher Elsevier
Peer Reviewed Peer Reviewed


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