| First Author | Su XW | Year | 2016 |
| Journal | Muscle Nerve | Volume | 54 |
| Issue | 2 | Pages | 284-91 |
| PubMed ID | 26799243 | Mgi Jnum | J:357704 |
| Mgi Id | MGI:7763904 | Doi | 10.1002/mus.25048 |
| Citation | Su XW, et al. (2016) Statins accelerate disease progression and shorten survival in SOD1(G93A) mice. Muscle Nerve 54(2):284-91 |
| abstractText | INTRODUCTION: HMG-CoA reductase inhibitors (statins) and H63D HFE polymorphism may modify amyotrophic lateral sclerosis (ALS). We hypothesized that statins worsen phenotype in ALS mice, dependent on HFE genotype. METHODS: Mice harboring SOD1(G93A) heterozygous for H67D Hfe (homologous to human H63D HFE) were administered simvastatin and/or coenzyme Q10, and were allowed to reach end stage. Disease progression was measured by grip strength. A separate group of animals was administered simvastatin and euthanized at the symptomatic 120-day time-point. Mitochondria from gastrocnemius muscle and lumbar spine were analyzed. RESULTS: Simvastatin and H67D Hfe accelerated disease progression. Simvastatin decreased survival. Coenzyme Q10 did not rescue statin-induced effects. Statins did not alter mitochondrial protein levels. CONCLUSIONS: Statins and Hfe genotype alter disease course in the ALS mouse model. Because the H63D HFE polymorphism is present in 30% of patients with ALS, studying disease progression in patients who receive statins, stratified for HFE genotype, may guide therapy. Muscle Nerve, 2016 Muscle Nerve 54: 284-291, 2016. |
