Guanabenz treatment improves Oculopharyngeal muscular dystrophy phenotype
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Summary
Modulation of protein folding regulation can be beneficial for OPMD and support the further development of guanabenz or its derivatives for treatment of O PMD in humans is supported.
- Type
- preprint
- Published
- 2018-07-24
- Cited by
- 0
- References
- 81
- Access
- Open access
- OpenAlex
- https://openalex.org/W2884349122
- Semantic Scholar
- https://api.semanticscholar.org/CorpusID:91444506
Keywords
Oculopharyngeal muscular dystrophy, Unfolded protein response, Cancer research, Phenotype, Endoplasmic reticulum
References
- Oculopharyngeal muscular dystrophy: recent advances in the understanding of the molecular pathogenic mechanisms and treatment strategies.
- Structural and Functional Analysis of the GADD34:PP1 eIF2α Phosphatase.
- Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophy
- Ca(2+) homeostasis and endoplasmic reticulum (ER) stress: An integrated view of calcium signaling.
- Mice conditionally lacking the Wolfram gene in pancreatic islet beta cells exhibit diabetes as a result of enhanced endoplasmic reticulum stress and apoptosis
- Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients
- Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant PABPN1 protein.
- A Drosophila model of oculopharyngeal muscular dystrophy reveals intrinsic toxicity of PABPN1
- Intracellular spiral inclusions in cerebral cell processes in Creutzfeldt-Jakob disease.
- Antihypertensive Drug Guanabenz Is Active In Vivo against both Yeast and Mammalian Prions
- The Human Nuclear Poly(A)-Binding Protein Promotes RNA Hyperadenylation and Decay
- Increased insulin demand promotes while pioglitazone prevents pancreatic beta cell apoptosis in Wfs1 knockout mice
- Mitochondrial Dysfunction Reveals the Role of mRNA Poly(A) Tail Regulation in Oculopharyngeal Muscular Dystrophy Pathogenesis
- Nuclear inclusion in oculopharyngeal dystrophy
- Chronic systemic therapy with low-dose morpholino oligomers ameliorates the pathology and normalizes locomotor behavior in mdx mice.
- Doxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic mice
- PABPN1 overexpression leads to upregulation of genes encoding nuclear proteins that are sequestered in oculopharyngeal muscular dystrophy nuclear inclusions.
- Clinical trial with guanabenz, a new antihypertensive agent.
- Homozygotes for oculopharyngeal muscular dystrophy have a severe form of the disease
- clusterProfiler: an R Package for Comparing Biological Themes Among Gene Clusters
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