ArticleNeuromuscular disorders : NMD2007
Upregulation of the creatine synthetic pathway in skeletal muscles of mature mdx mice.
Article in Neuromuscular disorders : NMD, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 25 citations in OpenAlex.
- Homoarginine, heart failure, and sudden cardiac death in haemodialysis patients.European journal of heart failure · 2011Trial
- Multi-modal tissue-aware graph neural network forbioRxiv : the preprint server for biology · 2026Article
- Creatine synthesis in the skeletal muscle: the times they are a-changin'.American journal of physiology. Endocrinology and metabolism · 2021Article
- Evolutionary expression differences of creatine synthesis-related genes: Implications for skeletal muscle metabolism in fish.Scientific reports · 2019Article
- Sil1-Mutant Mice Elucidate Chaperone Function in Neurological Disorders.Journal of neuromuscular diseases · 2016Article
- Differential Gene Expression Profiling of Dystrophic Dog Muscle after MuStem Cell Transplantation.PloS one · 2015Article
- ¹³C NMR metabolomics: applications at natural abundance.Analytical chemistry · 2014Article
- Creatine transporter (SLC6A8) knockout mice display an increased capacity for in vitro creatine biosynthesis in skeletal muscle.Frontiers in physiology · 2014Article
- Creatine metabolism differs between mammals and rainbow trout (Oncorhynchus mykiss).SpringerPlus · 2014Article
- AGAT knockout mice provide an opportunity to titrate tissue creatine content.The Journal of physiology · 2013Article
- Gene expression profiling of skeletal muscle of nursing piglets.International journal of biological sciences · 2010Article
- Transcriptomic analysis of dystrophin RNAi knockdown reveals a central role for dystrophin in muscle differentiation and contractile apparatus organization.BMC genomics · 2010Article
- Automated drug screening with contractile muscle tissue engineered from dystrophic myoblasts.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2009Article
- Molecular analysis of stem cells and their descendants during cell turnover and regeneration in the planarian Schmidtea mediterranea.Cell stem cell · 2008Article
- Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy.Neurobiology of disease · 2008Review
Corrections and comments
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Authors and funding
4 authors at 3 institutions in 2 countries.
Funding
Abstract
Duchenne muscular dystrophy (DMD) is a fatal neuromuscular human disease caused by dystrophin deficiency. The mdx mouse lacks dystrophin protein, yet does not exhibit the debilitating DMD phenotype. Investigating compensatory mechanisms in the mdx mouse may shed new insights into modifying DMD pathogenesis. This study targets two metabolic genes, guanidinoacetate methyltransferase (GAMT) and arginine:glycine amidinotransferase (AGAT) which are required for creatine synthesis. We show that GAMT and AGAT mRNA are up-regulated 5.4- and 1.9-fold respectively in adult mdx muscle compared to C57. In addition, GAMT protein expression is up-regulated at least 2.5-fold in five different muscles of mdx vs. control. Furthermore, we find GAMT immunoreactivity in up to 80% of mature mdx muscle fibers in addition to small regenerating fibers and rare revertants; while GAMT immunoreactivity is equal to background levels in all muscle fibers of mature C57 mice. The up-regulation of the creatine synthetic pathway may help maintain muscle creatine levels and limit cellular energy failure in leaky mdx skeletal muscles. These results may help better understand the mild phenotype of the mdx mouse and may offer new treatment horizons for DMD.
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