These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
2. Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice. McLoughlin HS; Moore LR; Chopra R; Komlo R; McKenzie M; Blumenstein KG; Zhao H; Kordasiewicz HB; Shakkottai VG; Paulson HL Ann Neurol; 2018 Jul; 84(1):64-77. PubMed ID: 29908063 [TBL] [Abstract][Full Text] [Related]
3. Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3. Shakkottai VG; do Carmo Costa M; Dell'Orco JM; Sankaranarayanan A; Wulff H; Paulson HL J Neurosci; 2011 Sep; 31(36):13002-14. PubMed ID: 21900579 [TBL] [Abstract][Full Text] [Related]
4. Growth hormone rescue cerebellar degeneration in SCA3 transgenic mice. Wu S; Liu K; Cheng W; Su S; Lin Y; Lin T; Cheng Y; Chang J; Wu Y; Liu C Biochem Biophys Res Commun; 2020 Aug; 529(2):467-473. PubMed ID: 32703453 [TBL] [Abstract][Full Text] [Related]
5. In vivo assessment of riluzole as a potential therapeutic drug for spinocerebellar ataxia type 3. Schmidt J; Schmidt T; Golla M; Lehmann L; Weber JJ; Hübener-Schmid J; Riess O J Neurochem; 2016 Jul; 138(1):150-62. PubMed ID: 26990650 [TBL] [Abstract][Full Text] [Related]
6. Polyglutamine-expanded ataxin-3 impairs long-term depression in Purkinje neurons of SCA3 transgenic mouse by inhibiting HAT and impairing histone acetylation. Chou AH; Chen YL; Hu SH; Chang YM; Wang HL Brain Res; 2014 Oct; 1583():220-9. PubMed ID: 25139423 [TBL] [Abstract][Full Text] [Related]
7. A combination of chlorzoxazone and folic acid improves recognition memory, anxiety and depression in SCA3-84Q mice. Marinina KS; Bezprozvanny IB; Egorova PA Hum Mol Genet; 2024 Aug; 33(16):1406-1419. PubMed ID: 38727562 [TBL] [Abstract][Full Text] [Related]
8. Pharmacological enhancement of retinoid-related orphan receptor α function mitigates spinocerebellar ataxia type 3 pathology. Watanave M; Hoshino C; Konno A; Fukuzaki Y; Matsuzaki Y; Ishitani T; Hirai H Neurobiol Dis; 2019 Jan; 121():263-273. PubMed ID: 30343032 [TBL] [Abstract][Full Text] [Related]
9. ASOs are an effective treatment for disease-associated oligodendrocyte signatures in premanifest and symptomatic SCA3 mice. Schuster KH; Zalon AJ; DiFranco DM; Putka AF; Stec NR; Jarrah SI; Naeem A; Haque Z; Zhang H; Guan Y; McLoughlin HS Mol Ther; 2024 May; 32(5):1359-1372. PubMed ID: 38429929 [TBL] [Abstract][Full Text] [Related]
10. Neuronal Atrophy Early in Degenerative Ataxia Is a Compensatory Mechanism to Regulate Membrane Excitability. Dell'Orco JM; Wasserman AH; Chopra R; Ingram MA; Hu YS; Singh V; Wulff H; Opal P; Orr HT; Shakkottai VG J Neurosci; 2015 Aug; 35(32):11292-307. PubMed ID: 26269637 [TBL] [Abstract][Full Text] [Related]
11. Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes. Ramani B; Panwar B; Moore LR; Wang B; Huang R; Guan Y; Paulson HL Hum Mol Genet; 2017 Sep; 26(17):3362-3374. PubMed ID: 28854700 [TBL] [Abstract][Full Text] [Related]
12. A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in Oligodendrocytes. Haas E; Incebacak RD; Hentrich T; Huridou C; Schmidt T; Casadei N; Maringer Y; Bahl C; Zimmermann F; Mills JD; Aronica E; Riess O; Schulze-Hentrich JM; Hübener-Schmid J Mol Neurobiol; 2022 Jan; 59(1):495-522. PubMed ID: 34716557 [TBL] [Abstract][Full Text] [Related]
14. A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD. Switonski PM; Szlachcic WJ; Krzyzosiak WJ; Figiel M Neurobiol Dis; 2015 Jan; 73():174-88. PubMed ID: 25301414 [TBL] [Abstract][Full Text] [Related]
15. Antisense oligonucleotide therapy rescues aggresome formation in a novel spinocerebellar ataxia type 3 human embryonic stem cell line. Moore LR; Keller L; Bushart DD; Delatorre RG; Li D; McLoughlin HS; do Carmo Costa M; Shakkottai VG; Smith GD; Paulson HL Stem Cell Res; 2019 Aug; 39():101504. PubMed ID: 31374463 [TBL] [Abstract][Full Text] [Related]
16. Mutant ataxin-3 with an abnormally expanded polyglutamine chain disrupts dendritic development and metabotropic glutamate receptor signaling in mouse cerebellar Purkinje cells. Konno A; Shuvaev AN; Miyake N; Miyake K; Iizuka A; Matsuura S; Huda F; Nakamura K; Yanagi S; Shimada T; Hirai H Cerebellum; 2014 Feb; 13(1):29-41. PubMed ID: 23955261 [TBL] [Abstract][Full Text] [Related]
18. Far-infrared Radiation Improves Motor Dysfunction and Neuropathology in Spinocerebellar Ataxia Type 3 Mice. Liu SW; Chang JC; Chuang SF; Liu KH; Cheng WL; Chang HJ; Chang HS; Lin TT; Hsieh CL; Lin WY; Hsieh M; Kuo SJ; Liu CS Cerebellum; 2019 Feb; 18(1):22-32. PubMed ID: 29725949 [TBL] [Abstract][Full Text] [Related]