Related Experiment Videos
Serum and organ indices of the mdx dystrophic mouse
G A Brazeau1, M Mathew, R K Entrikin
1Department of Pharmaceutics, College of Pharmacy, University of Houston, TX 77030.
Abstract:
Duchenne muscular dystrophy (DMD) is a fatal disease for which there is no effective treatment. The cause of death in patients with DMD is often cardiovascular and pulmonary dysfunction. This clinical observation, combined with experimental findings, suggests that other non-muscle organ systems may be affected in the dystrophic disease state. To test this hypothesis, the present study investigated liver and kidney function in the mdx mouse. Serum chemistries and the hepatic cytochrome P-450 system in normal and dystrophic mdx mice were investigated at two different ages. Increases in serum lactate dehydrogenase (LDH), alkaline phosphatase (AP), aspartate transaminase (AST), and cholesterol levels, combined with an increase in liver weight and a decrease in cytochrome P-450, suggests the possibility of hepatic dysfunction. Increases in serum uric acid and phosphorus, and decreased kidney weight suggest hepatic dysfunction.
Insights
Duchenne muscular dystrophy (DMD) affects more than muscles, impacting liver and kidney function. Research in mdx mice shows signs of hepatic and renal dysfunction in this fatal disease.
Area of Science:
- Biochemistry
- Pathology
- Genetics
Background:
- Duchenne muscular dystrophy (DMD) is a fatal genetic disorder primarily affecting skeletal muscle.
- Cardiovascular and pulmonary complications are leading causes of mortality in DMD patients.
- Emerging evidence suggests potential involvement of non-muscle organs in the dystrophic disease process.
Purpose of the Study:
- To investigate potential hepatic and renal dysfunction in the mdx mouse model of DMD.
- To assess serum chemistry profiles and hepatic cytochrome P-450 system activity in dystrophic mice.
Main Methods:
- Comparison of serum chemistries (LDH, AP, AST, cholesterol, uric acid, phosphorus) between normal and mdx mice at two different ages.
- Measurement of liver weight and hepatic cytochrome P-450 levels.
- Assessment of kidney weight.
Main Results:
- mdx mice exhibited elevated serum levels of LDH, AP, AST, cholesterol, uric acid, and phosphorus compared to controls.
- Liver weight was increased, while hepatic cytochrome P-450 levels were decreased in mdx mice.
- Kidney weight was decreased in mdx mice, suggesting potential renal impairment.
Conclusions:
- The study provides evidence for hepatic dysfunction in mdx mice, indicated by altered serum chemistries, increased liver weight, and reduced cytochrome P-450.
- Findings suggest potential kidney dysfunction in mdx mice, evidenced by elevated uric acid and phosphorus, and decreased kidney weight.
- These results highlight the systemic nature of DMD, extending beyond muscle pathology to affect vital organs like the liver and kidneys.