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Multi-exon Skipping Using Cocktail Antisense Oligonucleotides in the Canine X-linked Muscular Dystrophy
Published on: May 24, 2016
Immunophenotyping lymphocyte and acute phase proteins in canine X-linked muscular dystrophy
Dilayla K DE Abreu1, Janaina M Monteiro2, Carolina C Souza1
1Departamento de Cirurgia, Faculdade de Medicina Veterinária e Zootecnia/FMVA, Universidade de São Paulo/USP, Av. Prof. Dr. Orlando Marques de Paiva, 87, Cidade Universitária, 05508-270 São Paulo, SP, Brazil.
Insights
This study analyzed lymphocyte subpopulations and serum proteins in Golden Retriever dogs with Duchenne Muscular Dystrophy (GRMD). Older GRMD dogs showed higher CD4+ and CD5+ lymphocytes and altered acute phase proteins, indicating disease progression.
Area of Science:
- Veterinary Medicine
- Immunology
- Biochemistry
Background:
- Duchenne Muscular Dystrophy (DMD) is a severe X-linked muscle-wasting disease.
- The Golden Retriever Muscular Dystrophy (GRMD) model is a valuable tool for studying DMD.
- Understanding immune and protein changes in GRMD is crucial for translational research.
Purpose of the Study:
- To quantify CD4, CD5, and CD8 lymphocyte subpopulations.
- To standardize serum electrophoretic profiles.
- To investigate their contribution to the pathological process in GRMD dogs compared to healthy controls.
Main Methods:
- Analysis of umbilical cord blood from GRMD and healthy Golden Retrievers (GR) at different age groups (II: 2-3 months, III: >1 year).
- Flow cytometry for lymphocyte subpopulation quantification.
- Serum electrophoresis to identify and quantify proteins.
Main Results:
- No significant differences in CD8+ lymphocyte subpopulations were found between groups.
- CD4+ and CD5+ lymphocyte subpopulations were significantly higher in older GRMD dogs (GRMD III) compared to age-matched controls (GR III).
- Serum protein concentrations were lower in the youngest groups (GR I and GRMD I) and acute phase proteins worsened with age in GRMD dogs.
Conclusions:
- Age-related changes in CD4+ and CD5+ lymphocytes and serum proteins are evident in the GRMD model.
- These findings enhance the understanding of the GRMD model's utility for DMD research.
- The study provides translational insights into DMD pathogenesis and potential therapeutic targets.
Abstract:
Duchenne Muscular Dystrophy (DMD) is the most common X-linked muscular disease affecting humans. The Golden Retriever Muscular Dystrophy model (GRMD) is considerthe most suitable for several studies. This assay aims to quantify lymphocyte subpopulations CD4, CD5, and CD8, and standardize, the serum electrophoretic profile, to understand their contribution to the pathologic process in normal Golden Retriever dogs (GR group) and dystrophic´s (GRMD group), through the umbilical cord blood, in dogs aged from 2 to 3 months (GR II and GRMD II), and in dogs over 1 year of age (GR III and GRMD III). No significant differences were observed between the CD8+ lymphocyte subpopulations of the groups studied. The CD4+ and CD5+ lymphocyte subpopulations were significantly higher in the GRMD III group compared to the GR III group. Twenty-two different proteins in the gel were identified. The serum concentrations of the proteins belonging to the GR I and GRMD I groups were significantly lower than those of the other groups. We show that expression of acute phase proteins are worst during the aging of the dogs. We hope to expand knowledge to better understand the GRMD model and the translational data.
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