R01 NS079775/NS/NINDS NIH HHS/United States[Grants and Funding] - Search Results

Year	Number of Results
2014	6
2015	2
2016	3
2017	3
2018	1
2019	1
2024	0

1

Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS) Motor Dysfunction Modeled in Mice.

Foote M, Arque G, Berman RF, Santos M. Foote M, et al. Cerebellum. 2016 Oct;15(5):611-22. doi: 10.1007/s12311-016-0797-6. Cerebellum. 2016. PMID: 27255703 Free PMC article. Review.

2

Potential pathogenic mechanisms underlying Fragile X Tremor Ataxia Syndrome: RAN translation and/or RNA gain-of-function?

Boivin M, Willemsen R, Hukema RK, Sellier C. Boivin M, et al. Eur J Med Genet. 2018 Nov;61(11):674-679. doi: 10.1016/j.ejmg.2017.11.001. Epub 2017 Dec 6. Eur J Med Genet. 2018. PMID: 29223504 Review.

3

What has been learned from mouse models of the Fragile X Premutation and Fragile X-associated tremor/ataxia syndrome?

Foote MM, Careaga M, Berman RF. Foote MM, et al. Clin Neuropsychol. 2016 Aug;30(6):960-72. doi: 10.1080/13854046.2016.1158254. Epub 2016 Jun 29. Clin Neuropsychol. 2016. PMID: 27355912 Free PMC article. Review.

4

Mouse models of the fragile X premutation and fragile X-associated tremor/ataxia syndrome.

Berman RF, Buijsen RA, Usdin K, Pintado E, Kooy F, Pretto D, Pessah IN, Nelson DL, Zalewski Z, Charlet-Bergeurand N, Willemsen R, Hukema RK. Berman RF, et al. J Neurodev Disord. 2014;6(1):25. doi: 10.1186/1866-1955-6-25. Epub 2014 Jul 30. J Neurodev Disord. 2014. PMID: 25136376 Free PMC article. Review.

5

CNS expression of murine fragile X protein (FMRP) as a function of CGG-repeat size.

Ludwig AL, Espinal GM, Pretto DI, Jamal AL, Arque G, Tassone F, Berman RF, Hagerman PJ. Ludwig AL, et al. Hum Mol Genet. 2014 Jun 15;23(12):3228-38. doi: 10.1093/hmg/ddu032. Epub 2014 Jan 23. Hum Mol Genet. 2014. PMID: 24463622 Free PMC article.

6

Reduced activity-dependent protein levels in a mouse model of the fragile X premutation.

von Leden RE, Curley LC, Greenberg GD, Hunsaker MR, Willemsen R, Berman RF. von Leden RE, et al. Neurobiol Learn Mem. 2014 Mar;109:160-8. doi: 10.1016/j.nlm.2014.01.011. Epub 2014 Jan 23. Neurobiol Learn Mem. 2014. PMID: 24462720 Free PMC article.

7

Selective rescue of heightened anxiety but not gait ataxia in a premutation 90CGG mouse model of Fragile X-associated tremor/ataxia syndrome.

Castro H, Kul E, Buijsen RAM, Severijnen LWFM, Willemsen R, Hukema RK, Stork O, Santos M. Castro H, et al. Hum Mol Genet. 2017 Jun 1;26(11):2133-2145. doi: 10.1093/hmg/ddx108. Hum Mol Genet. 2017. PMID: 28369393 Free PMC article.

8

Reversibility of neuropathology and motor deficits in an inducible mouse model for FXTAS.

Hukema RK, Buijsen RA, Schonewille M, Raske C, Severijnen LA, Nieuwenhuizen-Bakker I, Verhagen RF, van Dessel L, Maas A, Charlet-Berguerand N, De Zeeuw CI, Hagerman PJ, Berman RF, Willemsen R. Hukema RK, et al. Hum Mol Genet. 2015 Sep 1;24(17):4948-57. doi: 10.1093/hmg/ddv216. Epub 2015 Jun 9. Hum Mol Genet. 2015. PMID: 26060190 Free PMC article.

9

Presence of inclusions positive for polyglycine containing protein, FMRpolyG, indicates that repeat-associated non-AUG translation plays a role in fragile X-associated primary ovarian insufficiency.

Buijsen RA, Visser JA, Kramer P, Severijnen EA, Gearing M, Charlet-Berguerand N, Sherman SL, Berman RF, Willemsen R, Hukema RK. Buijsen RA, et al. Hum Reprod. 2016 Jan;31(1):158-68. doi: 10.1093/humrep/dev280. Epub 2015 Nov 3. Hum Reprod. 2016. PMID: 26537920 Free PMC article.

10

Induced expression of expanded CGG RNA causes mitochondrial dysfunction in vivo.

Hukema RK, Buijsen RA, Raske C, Severijnen LA, Nieuwenhuizen-Bakker I, Minneboo M, Maas A, de Crom R, Kros JM, Hagerman PJ, Berman RF, Willemsen R. Hukema RK, et al. Cell Cycle. 2014;13(16):2600-8. doi: 10.4161/15384101.2014.943112. Cell Cycle. 2014. PMID: 25486200 Free PMC article.

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