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Items: 1 to 20 of 29

1.

Broad distribution of ataxin 1 silencing in rhesus cerebella for spinocerebellar ataxia type 1 therapy.

Keiser MS, Kordower JH, Gonzalez-Alegre P, Davidson BL.

Brain. 2015 Dec;138(Pt 12):3555-66. doi: 10.1093/brain/awv292. Epub 2015 Oct 21.

PMID:
26490326
2.

Pumilio1 haploinsufficiency leads to SCA1-like neurodegeneration by increasing wild-type Ataxin1 levels.

Gennarino VA, Singh RK, White JJ, De Maio A, Han K, Kim JY, Jafar-Nejad P, di Ronza A, Kang H, Sayegh LS, Cooper TA, Orr HT, Sillitoe RV, Zoghbi HY.

Cell. 2015 Mar 12;160(6):1087-98. doi: 10.1016/j.cell.2015.02.012.

3.

A Coding Variant of ANO10, Affecting Volume Regulation of Macrophages, Is Associated with Borrelia Seropositivity.

Hammer C, Wanitchakool P, Sirianant L, Papiol S, Monnheimer M, Faria D, Ousingsawat J, Schramek N, Schmitt C, Margos G, Michel A, Kraiczy P, Pawlita M, Schreiber R, Schulz TF, Fingerle V, Tumani H, Ehrenreich H, Kunzelmann K.

Mol Med. 2015 Feb 23;21:26-37. doi: 10.2119/molmed.2014.00219.

4.

Oligonucleotide-based strategies to combat polyglutamine diseases.

Fiszer A, Krzyzosiak WJ.

Nucleic Acids Res. 2014 Jun;42(11):6787-810. doi: 10.1093/nar/gku385. Epub 2014 May 21. Review.

5.

Beyond the glutamine expansion: influence of posttranslational modifications of ataxin-1 in the pathogenesis of spinocerebellar ataxia type 1.

Ju H, Kokubu H, Lim J.

Mol Neurobiol. 2014 Dec;50(3):866-74. doi: 10.1007/s12035-014-8703-z. Epub 2014 Apr 22. Review.

6.

Broad therapeutic benefit after RNAi expression vector delivery to deep cerebellar nuclei: implications for spinocerebellar ataxia type 1 therapy.

Keiser MS, Boudreau RL, Davidson BL.

Mol Ther. 2014 Mar;22(3):588-95. doi: 10.1038/mt.2013.279. Epub 2013 Dec 12.

7.

Neuroprotective function of 14-3-3 proteins in neurodegeneration.

Shimada T, Fournier AE, Yamagata K.

Biomed Res Int. 2013;2013:564534. doi: 10.1155/2013/564534. Epub 2013 Dec 2. Review.

8.

Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias.

Matilla-Dueñas A, Ashizawa T, Brice A, Magri S, McFarland KN, Pandolfo M, Pulst SM, Riess O, Rubinsztein DC, Schmidt J, Schmidt T, Scoles DR, Stevanin G, Taroni F, Underwood BR, Sánchez I.

Cerebellum. 2014 Apr;13(2):269-302. doi: 10.1007/s12311-013-0539-y.

9.

Polyglutamine disease toxicity is regulated by Nemo-like kinase in spinocerebellar ataxia type 1.

Ju H, Kokubu H, Todd TW, Kahle JJ, Kim S, Richman R, Chirala K, Orr HT, Zoghbi HY, Lim J.

J Neurosci. 2013 May 29;33(22):9328-36. doi: 10.1523/JNEUROSCI.3465-12.2013.

10.

RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1.

Park J, Al-Ramahi I, Tan Q, Mollema N, Diaz-Garcia JR, Gallego-Flores T, Lu HC, Lagalwar S, Duvick L, Kang H, Lee Y, Jafar-Nejad P, Sayegh LS, Richman R, Liu X, Gao Y, Shaw CA, Arthur JS, Orr HT, Westbrook TF, Botas J, Zoghbi HY.

Nature. 2013 Jun 20;498(7454):325-31. doi: 10.1038/nature12204. Epub 2013 May 29.

11.

RNAi or overexpression: alternative therapies for Spinocerebellar Ataxia Type 1.

Keiser MS, Geoghegan JC, Boudreau RL, Lennox KA, Davidson BL.

Neurobiol Dis. 2013 Aug;56:6-13. doi: 10.1016/j.nbd.2013.04.003. Epub 2013 Apr 10.

12.

Ataxin1L is a regulator of HSC function highlighting the utility of cross-tissue comparisons for gene discovery.

Kahle JJ, Souroullas GP, Yu P, Zohren F, Lee Y, Shaw CA, Zoghbi HY, Goodell MA.

PLoS Genet. 2013 Mar;9(3):e1003359. doi: 10.1371/journal.pgen.1003359. Epub 2013 Mar 28.

13.

Ataxin-2-like is a regulator of stress granules and processing bodies.

Kaehler C, Isensee J, Nonhoff U, Terrey M, Hucho T, Lehrach H, Krobitsch S.

PLoS One. 2012;7(11):e50134. doi: 10.1371/journal.pone.0050134. Epub 2012 Nov 27.

14.

Mouse models of polyglutamine diseases in therapeutic approaches: review and data table. Part II.

Switonski PM, Szlachcic WJ, Gabka A, Krzyzosiak WJ, Figiel M.

Mol Neurobiol. 2012 Oct;46(2):430-66. doi: 10.1007/s12035-012-8316-3. Epub 2012 Sep 4. Review.

15.

The Capicua repressor--a general sensor of RTK signaling in development and disease.

Jiménez G, Shvartsman SY, Paroush Z.

J Cell Sci. 2012 Mar 15;125(Pt 6):1383-91. doi: 10.1242/jcs.092965. Review.

16.

Cell biology of spinocerebellar ataxia.

Orr HT.

J Cell Biol. 2012 Apr 16;197(2):167-77. doi: 10.1083/jcb.201105092. Review.

17.

ATXN1 protein family and CIC regulate extracellular matrix remodeling and lung alveolarization.

Lee Y, Fryer JD, Kang H, Crespo-Barreto J, Bowman AB, Gao Y, Kahle JJ, Hong JS, Kheradmand F, Orr HT, Finegold MJ, Zoghbi HY.

Dev Cell. 2011 Oct 18;21(4):746-57. doi: 10.1016/j.devcel.2011.08.017.

18.

Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias.

Ingram MA, Orr HT, Clark HB.

Brain Res Bull. 2012 May 1;88(1):33-42. doi: 10.1016/j.brainresbull.2011.07.016. Epub 2011 Jul 23. Review.

19.

Huntington's Disease.

Finkbeiner S.

Cold Spring Harb Perspect Biol. 2011 Jun 1;3(6). pii: a007476. doi: 10.1101/cshperspect.a007476. Review.

20.

Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3epsilon haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration.

Jafar-Nejad P, Ward CS, Richman R, Orr HT, Zoghbi HY.

Proc Natl Acad Sci U S A. 2011 Feb 1;108(5):2142-7. doi: 10.1073/pnas.1018748108. Epub 2011 Jan 18.

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