FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2896025068> ?p ?o ?g. }

• W2896025068 endingPage "273" @default.
• W2896025068 startingPage "263" @default.
• W2896025068 abstract "Cerebellar Purkinje cells (PCs) are the sole output neurons of the cerebellar cortex, and damage to PCs results in motor deficits. Spinocerebellar ataxia type 3 (SCA3, also known as Machado–Joseph disease), a hereditary neurodegenerative disease, is caused by an abnormal expansion of the polyglutamine tract in the causative ATXN3 protein. SCA3 affects a wide range of cells in the central nervous system, including those in the cerebellum. To unravel SCA3 pathology, we used adeno-associated virus serotype 9 (AAV9) vectors to express full-length ATXN3 with an abnormally expanded 89 polyglutamine stretch (ATXN3[Q89]) in cerebellar neurons of mature wild-type mice. Mice expressing ATXN3[Q89] exhibited motor impairment in a manner dependent on the viral titer. Immunohistochemistry of the cerebellum showed ubiquitinated nuclear aggregates in PCs; degeneration of PC dendrites; and a significant decrease in multiple proteins including retinoid-related orphan receptor α (RORα), a transcription factor, and type 1 metabotropic glutamate receptor (mGluR1) signaling molecules. Patch clamp analysis of ATXN3[Q89]-expressing PCs revealed marked defects in mGluR1 signaling. Notably, the emergence of behavioral, morphological, and functional defects was inhibited by a single injection of SR1078, an RORα/γ agonist. These results suggest that RORα plays a key role in mutant ATXN3-mediated aberrant phenotypes and that the pharmacological enhancement of RORα could function as a method for therapeutic intervention in SCA3." @default.
• W2896025068 created "2018-10-26" @default.
• W2896025068 creator A5001341469 @default.
• W2896025068 creator A5030392495 @default.
• W2896025068 creator A5051911822 @default.
• W2896025068 creator A5061243316 @default.
• W2896025068 creator A5073680568 @default.
• W2896025068 creator A5083968498 @default.
• W2896025068 creator A5088851512 @default.
• W2896025068 date "2019-01-01" @default.
• W2896025068 modified "2023-09-25" @default.
• W2896025068 title "Pharmacological enhancement of retinoid-related orphan receptor α function mitigates spinocerebellar ataxia type 3 pathology" @default.
• W2896025068 cites W1180737757 @default.
• W2896025068 cites W1514826128 @default.
• W2896025068 cites W1566613423 @default.
• W2896025068 cites W1966809675 @default.
• W2896025068 cites W1967784729 @default.
• W2896025068 cites W1968257708 @default.
• W2896025068 cites W1975006022 @default.
• W2896025068 cites W1986865371 @default.
• W2896025068 cites W1990676315 @default.
• W2896025068 cites W1992203015 @default.
• W2896025068 cites W1992458598 @default.
• W2896025068 cites W2000366854 @default.
• W2896025068 cites W2009077902 @default.
• W2896025068 cites W2021429993 @default.
• W2896025068 cites W2037057300 @default.
• W2896025068 cites W2038610378 @default.
• W2896025068 cites W2043888873 @default.
• W2896025068 cites W2054694814 @default.
• W2896025068 cites W2055999104 @default.
• W2896025068 cites W2065329118 @default.
• W2896025068 cites W2068988203 @default.
• W2896025068 cites W2073012734 @default.
• W2896025068 cites W2088772156 @default.
• W2896025068 cites W2088872333 @default.
• W2896025068 cites W2096105837 @default.
• W2896025068 cites W2101347364 @default.
• W2896025068 cites W2106229319 @default.
• W2896025068 cites W2109902738 @default.
• W2896025068 cites W2128094872 @default.
• W2896025068 cites W2143287512 @default.
• W2896025068 cites W2167795186 @default.
• W2896025068 cites W2170868833 @default.
• W2896025068 cites W2170970652 @default.
• W2896025068 cites W2214952879 @default.
• W2896025068 cites W2255469197 @default.
• W2896025068 cites W2256579886 @default.
• W2896025068 cites W2313162241 @default.
• W2896025068 cites W2328109216 @default.
• W2896025068 cites W2345315856 @default.
• W2896025068 cites W2345677745 @default.
• W2896025068 cites W2505580917 @default.
• W2896025068 cites W2588443411 @default.
• W2896025068 cites W2739993389 @default.
• W2896025068 cites W2769590953 @default.
• W2896025068 cites W2791741887 @default.
• W2896025068 cites W2796598047 @default.
• W2896025068 cites W757801746 @default.
• W2896025068 doi "https://doi.org/10.1016/j.nbd.2018.10.014" @default.
• W2896025068 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30343032" @default.
• W2896025068 hasPublicationYear "2019" @default.
• W2896025068 type Work @default.
• W2896025068 sameAs 2896025068 @default.
• W2896025068 citedByCount "16" @default.
• W2896025068 countsByYear W28960250682019 @default.
• W2896025068 countsByYear W28960250682020 @default.
• W2896025068 countsByYear W28960250682021 @default.
• W2896025068 countsByYear W28960250682022 @default.
• W2896025068 countsByYear W28960250682023 @default.
• W2896025068 crossrefType "journal-article" @default.
• W2896025068 hasAuthorship W2896025068A5001341469 @default.
• W2896025068 hasAuthorship W2896025068A5030392495 @default.
• W2896025068 hasAuthorship W2896025068A5051911822 @default.
• W2896025068 hasAuthorship W2896025068A5061243316 @default.
• W2896025068 hasAuthorship W2896025068A5073680568 @default.
• W2896025068 hasAuthorship W2896025068A5083968498 @default.
• W2896025068 hasAuthorship W2896025068A5088851512 @default.
• W2896025068 hasConcept C104317684 @default.
• W2896025068 hasConcept C142724271 @default.
• W2896025068 hasConcept C143065580 @default.
• W2896025068 hasConcept C169760540 @default.
• W2896025068 hasConcept C170493617 @default.
• W2896025068 hasConcept C207951395 @default.
• W2896025068 hasConcept C2776925932 @default.
• W2896025068 hasConcept C2777616661 @default.
• W2896025068 hasConcept C2778107364 @default.
• W2896025068 hasConcept C2779134260 @default.
• W2896025068 hasConcept C2779161069 @default.
• W2896025068 hasConcept C2779500118 @default.
• W2896025068 hasConcept C2779652256 @default.
• W2896025068 hasConcept C2780906641 @default.
• W2896025068 hasConcept C2781427258 @default.
• W2896025068 hasConcept C40767141 @default.
• W2896025068 hasConcept C49051014 @default.
• W2896025068 hasConcept C54355233 @default.
• W2896025068 hasConcept C61174792 @default.
• W2896025068 hasConcept C71924100 @default.

• next