Sopher BL - Search Results

1

Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients.

Bennett CL, Dastidar SG, Ling SC, Malik B, Ashe T, Wadhwa M, Miller DB, Lee C, Mitchell MB, van Es MA, Grunseich C, Chen Y, Sopher BL, Greensmith L, Cleveland DW, La Spada AR. Bennett CL, et al. Among authors: sopher bl. Acta Neuropathol. 2018 Sep;136(3):425-443. doi: 10.1007/s00401-018-1852-9. Epub 2018 May 3. Acta Neuropathol. 2018. PMID: 29725819 Free PMC article.

2

In vivo expansion of trinucleotide repeats yields plasmid and YAC constructs for targeting and transgenesis.

Sopher BL, Myrick SB, Hong JY, Smith AC, La Spada AR. Sopher BL, et al. Gene. 2000 Dec 31;261(2):383-90. doi: 10.1016/s0378-1119(00)00508-4. Gene. 2000. PMID: 11167027

3

Genomic organization, chromosome location, and expression analysis of mouse beta-synuclein, a candidate for involvement in neurodegeneration.

Sopher BL, Koszdin KL, McClain ME, Myrick SB, Martinez RA, Smith AC, La Spada AR. Sopher BL, et al. Cytogenet Cell Genet. 2001;93(1-2):117-23. doi: 10.1159/000056962. Cytogenet Cell Genet. 2001. PMID: 11474193

4

Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7.

La Spada AR, Fu YH, Sopher BL, Libby RT, Wang X, Li LY, Einum DD, Huang J, Possin DE, Smith AC, Martinez RA, Koszdin KL, Treuting PM, Ware CB, Hurley JB, Ptácek LJ, Chen S. La Spada AR, et al. Among authors: sopher bl. Neuron. 2001 Sep 27;31(6):913-27. doi: 10.1016/s0896-6273(01)00422-6. Neuron. 2001. PMID: 11580893 Free article.

5

Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.

Garden GA, Libby RT, Fu YH, Kinoshita Y, Huang J, Possin DE, Smith AC, Martinez RA, Fine GC, Grote SK, Ware CB, Einum DD, Morrison RS, Ptacek LJ, Sopher BL, La Spada AR. Garden GA, et al. Among authors: sopher bl. J Neurosci. 2002 Jun 15;22(12):4897-905. doi: 10.1523/JNEUROSCI.22-12-04897.2002. J Neurosci. 2002. PMID: 12077187 Free PMC article.

6

Interference of Crx-dependent transcription by ataxin-7 involves interaction between the glutamine regions and requires the ataxin-7 carboxy-terminal region for nuclear localization.

Chen S, Peng GH, Wang X, Smith AC, Grote SK, Sopher BL, La Spada AR. Chen S, et al. Among authors: sopher bl. Hum Mol Genet. 2004 Jan 1;13(1):53-67. doi: 10.1093/hmg/ddh005. Epub 2003 Nov 12. Hum Mol Genet. 2004. PMID: 14613968

7

Androgen receptor YAC transgenic mice recapitulate SBMA motor neuronopathy and implicate VEGF164 in the motor neuron degeneration.

Sopher BL, Thomas PS Jr, LaFevre-Bernt MA, Holm IE, Wilke SA, Ware CB, Jin LW, Libby RT, Ellerby LM, La Spada AR. Sopher BL, et al. Neuron. 2004 Mar 4;41(5):687-99. doi: 10.1016/s0896-6273(04)00082-0. Neuron. 2004. PMID: 15003169 Free article.

8

The Purkinje cell degeneration 5J mutation is a single amino acid insertion that destabilizes Nna1 protein.

Chakrabarti L, Neal JT, Miles M, Martinez RA, Smith AC, Sopher BL, La Spada AR. Chakrabarti L, et al. Among authors: sopher bl. Mamm Genome. 2006 Feb;17(2):103-10. doi: 10.1007/s00335-005-0096-x. Epub 2006 Feb 7. Mamm Genome. 2006. PMID: 16465590

9

Efficient recombination-based methods for bacterial artificial chromosome fusion and mutagenesis.

Sopher BL, La Spada AR. Sopher BL, et al. Gene. 2006 Apr 12;371(1):136-43. doi: 10.1016/j.gene.2005.11.034. Epub 2006 Feb 17. Gene. 2006. PMID: 16487669

10

Loss of endogenous androgen receptor protein accelerates motor neuron degeneration and accentuates androgen insensitivity in a mouse model of X-linked spinal and bulbar muscular atrophy.

Thomas PS Jr, Fraley GS, Damian V, Woodke LB, Zapata F, Sopher BL, Plymate SR, La Spada AR. Thomas PS Jr, et al. Among authors: sopher bl. Hum Mol Genet. 2006 Jul 15;15(14):2225-38. doi: 10.1093/hmg/ddl148. Epub 2006 Jun 13. Hum Mol Genet. 2006. PMID: 16772330

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