Orr HT - Search Results

1

Cross-species genetic screens identify transglutaminase 5 as a regulator of polyglutamine-expanded ataxin-1.

Lee WS, Al-Ramahi I, Jeong HH, Jang Y, Lin T, Adamski CJ, Lavery LA, Rath S, Richman R, Bondar VV, Alcala E, Revelli JP, Orr HT, Liu Z, Botas J, Zoghbi HY. Lee WS, et al. Among authors: orr ht. J Clin Invest. 2022 May 2;132(9):e156616. doi: 10.1172/JCI156616. J Clin Invest. 2022. PMID: 35499073 Free PMC article.

2

Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes.

Bowman AB, Lam YC, Jafar-Nejad P, Chen HK, Richman R, Samaco RC, Fryer JD, Kahle JJ, Orr HT, Zoghbi HY. Bowman AB, et al. Among authors: orr ht. Nat Genet. 2007 Mar;39(3):373-9. doi: 10.1038/ng1977. Epub 2007 Feb 18. Nat Genet. 2007. PMID: 17322884

3

Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1.

Zoghbi HY, Orr HT. Zoghbi HY, et al. Among authors: orr ht. J Biol Chem. 2009 Mar 20;284(12):7425-9. doi: 10.1074/jbc.R800041200. Epub 2008 Oct 28. J Biol Chem. 2009. PMID: 18957430 Free PMC article. Review.

4

PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in spinocerebellar ataxia type 1.

Bondar VV, Adamski CJ, Onur TS, Tan Q, Wang L, Diaz-Garcia J, Park J, Orr HT, Botas J, Zoghbi HY. Bondar VV, et al. Among authors: orr ht. Hum Mol Genet. 2018 Aug 15;27(16):2863-2873. doi: 10.1093/hmg/ddy200. Hum Mol Genet. 2018. PMID: 29860311 Free PMC article.

5

Dual targeting of brain region-specific kinases potentiates neurological rescue in Spinocerebellar ataxia type 1.

Lee WS, Lavery L, Rousseaux MWC, Rutledge EB, Jang Y, Wan YW, Wu SR, Kim W, Al-Ramahi I, Rath S, Adamski CJ, Bondar VV, Tewari A, Soleimani S, Mota S, Yalamanchili HK, Orr HT, Liu Z, Botas J, Zoghbi HY. Lee WS, et al. Among authors: orr ht. EMBO J. 2021 Apr 1;40(7):e106106. doi: 10.15252/embj.2020106106. Epub 2021 Mar 11. EMBO J. 2021. PMID: 33709453 Free PMC article.

6

Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice.

Skinner PJ, Vierra-Green CA, Emamian E, Zoghbi HY, Orr HT. Skinner PJ, et al. Among authors: orr ht. Neuromolecular Med. 2002;1(1):33-42. doi: 10.1385/NMM:1:1:33. Neuromolecular Med. 2002. PMID: 12025814

7

Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice.

Emamian ES, Kaytor MD, Duvick LA, Zu T, Tousey SK, Zoghbi HY, Clark HB, Orr HT. Emamian ES, et al. Among authors: orr ht. Neuron. 2003 May 8;38(3):375-87. doi: 10.1016/s0896-6273(03)00258-7. Neuron. 2003. PMID: 12741986 Free article.

8

Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.

Chen HK, Fernandez-Funez P, Acevedo SF, Lam YC, Kaytor MD, Fernandez MH, Aitken A, Skoulakis EM, Orr HT, Botas J, Zoghbi HY. Chen HK, et al. Among authors: orr ht. Cell. 2003 May 16;113(4):457-68. doi: 10.1016/s0092-8674(03)00349-0. Cell. 2003. PMID: 12757707 Free article.

9

Regional differences of somatic CAG repeat instability do not account for selective neuronal vulnerability in a knock-in mouse model of SCA1.

Watase K, Venken KJ, Sun Y, Orr HT, Zoghbi HY. Watase K, et al. Among authors: orr ht. Hum Mol Genet. 2003 Nov 1;12(21):2789-95. doi: 10.1093/hmg/ddg300. Epub 2003 Sep 2. Hum Mol Genet. 2003. PMID: 12952864

10

The effects of the polyglutamine repeat protein ataxin-1 on the UbL-UBA protein A1Up.

Riley BE, Xu Y, Zoghbi HY, Orr HT. Riley BE, et al. Among authors: orr ht. J Biol Chem. 2004 Oct 1;279(40):42290-301. doi: 10.1074/jbc.M406284200. Epub 2004 Jul 26. J Biol Chem. 2004. PMID: 15280365 Free article.

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