| http://purl.uniprot.org/citations/30231518 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/30231518 | http://www.w3.org/2000/01/rdf-schema#comment | "α-thalassemia X-linked intellectual disability (ATR-X) syndrome is caused by mutations in ATRX. An ATR-X model mouse lacking Atrx exon 2 displays phenotypes that resemble symptoms in the human intellectual disability: cognitive defects and abnormal dendritic spine formation. We herein target activation of sigma-1 receptor (Sig-1R) that can induce potent neuroprotective and neuroregenerative effects by promoting the activity of neurotrophic factors, such as brain-derived neurotrophic factor (BDNF). We demonstrated that treatment with SA4503, a potent activator of Sig-1R, reverses axonal development and dendritic spine abnormalities in cultured cortical neurons from ATR-X model mice. Moreover, the SA4503 treatment rescued cognitive deficits exhibited by the ATR-X model mice. We further found that significant decreases in the BDNF-protein level in the medial prefrontal cortex of ATR-X model mice were recovered with treatment of SA4503. These results indicate that the rescue of dendritic spine abnormalities through the activation of Sig-1R has a potential for post-diagnostic therapy in ATR-X syndrome."xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.org/dc/terms/identifier | "doi:10.3390/ijms19092811"xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/author | "Han F."xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/author | "Zhang C."xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/author | "Yamaguchi K."xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/author | "Fukunaga K."xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/author | "Shioda N."xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/author | "Yabuki Y."xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/date | "2018"xsd:gYear |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/name | "Int J Mol Sci"xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/pages | "E2811"xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/title | "SA4503, A Potent Sigma-1 Receptor Ligand, Ameliorates Synaptic Abnormalities and Cognitive Dysfunction in a Mouse Model of ATR-X Syndrome."xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://purl.uniprot.org/core/volume | "19"xsd:string |
| http://purl.uniprot.org/citations/30231518 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/30231518 |
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