| http://purl.uniprot.org/citations/37011198 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/37011198 | http://www.w3.org/2000/01/rdf-schema#comment | "RNA-binding protein (RBP) dysfunction is a fundamental hallmark of amyotrophic lateral sclerosis (ALS) and related neuromuscular disorders. Abnormal neuronal excitability is also a conserved feature in ALS patients and disease models, yet little is known about how activity-dependent processes regulate RBP levels and functions. Mutations in the gene encoding the RBP Matrin 3 (MATR3) cause familial disease, and MATR3 pathology has also been observed in sporadic ALS, suggesting a key role for MATR3 in disease pathogenesis. Here, we show that glutamatergic activity drives MATR3 degradation through an NMDA receptor-, Ca2+-, and calpain-dependent mechanism. The most common pathogenic MATR3 mutation renders it resistant to calpain degradation, suggesting a link between activity-dependent MATR3 regulation and disease. We also demonstrate that Ca2+ regulates MATR3 through a nondegradative process involving the binding of Ca2+/calmodulin to MATR3 and inhibition of its RNA-binding ability. These findings indicate that neuronal activity impacts both the abundance and function of MATR3, underscoring the effect of activity on RBPs and providing a foundation for further study of Ca2+-coupled regulation of RBPs implicated in ALS and related neurological diseases."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.org/dc/terms/identifier | "doi:10.1073/pnas.2206217120"xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/author | "Huang H."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/author | "Li X."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/author | "Wu J.J."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/author | "Shakkottai V.G."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/author | "Malik A.M."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/author | "Barmada S."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/author | "Doctrove Q.A."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/author | "Gillies C.A."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/author | "Tank E.H.M."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/date | "2023"xsd:gYear |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/name | "Proc Natl Acad Sci U S A"xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/pages | "e2206217120"xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/title | "Neuronal activity regulates Matrin 3 abundance and function in a calcium-dependent manner through calpain-mediated cleavage and calmodulin binding."xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://purl.uniprot.org/core/volume | "120"xsd:string |
| http://purl.uniprot.org/citations/37011198 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/37011198 |
| http://purl.uniprot.org/citations/37011198 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/37011198 |
| http://purl.uniprot.org/uniprot/#_A0A0R4J2E8-mappedCitation-37011198 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/37011198 |
| http://purl.uniprot.org/uniprot/#_B3KM87-mappedCitation-37011198 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/37011198 |
| http://purl.uniprot.org/uniprot/#_B4DRS1-mappedCitation-37011198 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/37011198 |
| http://purl.uniprot.org/uniprot/#_B4DF66-mappedCitation-37011198 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/37011198 |
| http://purl.uniprot.org/uniprot/#_P43243-mappedCitation-37011198 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/37011198 |
| http://purl.uniprot.org/uniprot/#_Q5CZA7-mappedCitation-37011198 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/37011198 |