Tight expression regulation of senataxin, linked to motor neuron disease and ataxia, is required to avert cell-cycle block and nucleolus disassembly

Autor Bennett, Craig L., Sopher, Bryce L., La Spada, Albert R.
Vydáno v Heliyon (2020)

Control of PERK eIF2α kinase activity by the endoplasmic reticulum stress-induced molecular chaperone P58(IPK)

Autor Yan, Wei, Frank, Christopher L., Korth, Marcus J., Sopher, Bryce L., Novoa, Isabel, Ron, David, Katze, Michael G.
Vydáno 2002

Alzheimer’s Presenilin Mutation Sensitizes Neural Cells to Apoptosis Induced by Trophic Factor Withdrawal and Amyloid β-Peptide: Involvement of Calcium and Oxyradicals

Autor Guo, Qing, Sopher, Bryce L., Furukawa, Katsutoshi, Pham, Dao G., Robinson, Nic, Martin, George M., Mattson, Mark P.
Vydáno v J Neurosci (1997)

The zinc-binding domain of Nna1 is required to prevent retinal photoreceptor loss and cerebellar ataxia in Purkinje cell degeneration (pcd) mice

Autor Chakrabarti, Lisa, Eng, Jeremiah, Martinez, Refugio A., Jackson, Stephen, Huang, Jing, Possin, Daniel E., Sopher, Bryce L., La Spada, Albert R.
Vydáno 2008

Neurotrophic factors [activity-dependent neurotrophic factor (ADNF) and basic fibroblast growth factor (bFGF)] interrupt excitotoxic neurodegenerative cascades promoted by a PS1 mu...

Autor Guo, Qing, Sebastian, Lois, Sopher, Bryce L., Miller, Miles W., Glazner, Gordon W., Ware, Carol B., Martin, George M., Mattson, Mark P.
Vydáno 1999

4E-BP1 Protects Neurons from Misfolded Protein Stress and Parkinson's Disease Toxicity by Inducing the Mitochondrial Unfolded Protein Response

Autor Dastidar, Somasish Ghosh, Pham, Michael T., Mitchell, Matthew B., Yeom, Steven G., Jordan, Sarah, Chang, Angela, Sopher, Bryce L., La Spada, Albert R.
Vydáno v J Neurosci (2020)

Spinocerebellar Ataxia Type 7 Cerebellar Disease Requires the Coordinated Action of Mutant Ataxin-7 in Neurons and Glia, and Displays Non-Cell-Autonomous Bergmann Glia Degeneration

Autor Furrer, Stephanie A., Mohanachandran, Mathini S., Waldherr, Sarah M., Chang, Christopher, Damian, Vincent A., Sopher, Bryce L., Garden, Gwenn A., La Spada, Albert R.
Vydáno 2011

Proteolytic cleavage of ataxin-7 promotes SCA7 retinal degeneration and neurological dysfunction

Autor Guyenet, Stephan J., Mookerjee, Shona S., Lin, Amy, Custer, Sara K., Chen, Sylvia F., Sopher, Bryce L., La Spada, Albert R., Ellerby, Lisa M.
Vydáno v Hum Mol Genet (2015)

Bax Interacting Factor-1 Promotes Survival and Mitochondrial Elongation in Neurons

Autor Wang, David B., Uo, Takuma, Kinoshita, Chizuru, Sopher, Bryce L., Lee, Rona J., Murphy, Sean P., Kinoshita, Yoshito, Garden, Gwenn A., Wang, Hong-Gang, Morrison, Richard S.
Vydáno 2014

Reduction of mutant ataxin-7 expression restores motor function and prevents cerebellar synaptic reorganization in a conditional mouse model of SCA7

Autor Furrer, Stephanie A., Waldherr, Sarah M., Mohanachandran, Mathini S., Baughn, Travis D., Nguyen, Kien-Thiet, Sopher, Bryce L., Damian, Vincent A., Garden, Gwenn A., La Spada, Albert R.
Vydáno 2013

Posttranslational Modification of Ataxin-7 at Lysine 257 Prevents Autophagy-Mediated Turnover of an N-Terminal Caspase-7 Cleavage Fragment

Autor Mookerjee, Shona, Papanikolaou, Theodora, Guyenet, Stephan J., Sampath, Vanitha, Lin, Amy, Vitelli, Cathy, DeGiacomo, Francesco, Sopher, Bryce L., Chen, Sylvia F., La Spada, Albert R., Ellerby, Lisa M.
Vydáno 2009

CTCF regulates ataxin-7 expression through promotion of a convergently transcribed, antisense non-coding RNA

Autor Sopher, Bryce L., Ladd, Paula D., Pineda, Victor V., Libby, Randell T., Sunkin, Susan M., Hurley, James B., Thienes, Cortlandt P., Gaasterland, Terry, Filippova, Galina N., La Spada, Albert R.
Vydáno 2011

Neuronal susceptibility to beta‐amyloid toxicity and ischemic injury involves histone deacetylase‐2 regulation of endophilin‐B1

Autor Wang, David B., Kinoshita, Chizuru, Kinoshita, Yoshito, Sopher, Bryce L., Uo, Takuma, Lee, Rona J., Kim, Joon Kyu, Murphy, Sean P., Dirk Keene, C., Garden, Gwenn A., Morrison, Richard S.
Vydáno v Brain Pathol (2018)

Polyglutamine-expanded androgen receptor interferes with TFEB to elicit pathological autophagy defects in SBMA

Autor Cortes, Constanza J., Miranda, Helen C., Frankowski, Harald, Batlevi, Yakup, Young, Jessica E., Le, Amy, Ivanov, Nishi, Sopher, Bryce L., Carromeu, Cassiano, Muotri, Alysson R., Garden, Gwenn A., La Spada, Albert R.
Vydáno 2014

Loss of endophilin-B1 exacerbates Alzheimer’s disease pathology

Autor Wang, David B., Kinoshita, Yoshito, Kinoshita, Chizuru, Uo, Takuma, Sopher, Bryce L., Cudaback, Eiron, Keene, C. Dirk, Bilousova, Tina, Gylys, Karen, Case, Amanda, Jayadev, Suman, Wang, Hong-Gang, Garden, Gwenn A., Morrison, Richard S.
Vydáno v Brain (2015)

Muscle expression of mutant androgen receptor protein accounts for systemic and motor neuron disease phenotypes in Spinal & Bulbar Muscular Atrophy

Autor Cortes, Constanza J., Ling, Shuo-Chien, Guo, Ling T., Hung, Gene, Tsunemi, Taiji, Ly, Linda, Tokunaga, Seiya, Lopez, Edith, Sopher, Bryce L., Bennett, C. Frank, Shelton, G. Diane, Cleveland, Don W., La Spada, Albert R.
Vydáno 2014

Polyglutamine-Expanded Androgen Receptor Truncation Fragments Activate a Bax-Dependent Apoptotic Cascade Mediated by DP5/Hrk

Autor Young, Jessica E., Garden, Gwenn A., Martinez, Refugio A., Tanaka, Fumiaki, Miguel Sandoval, C., Smith, Annette C., Sopher, Bryce L., Lin, Amy, Fischbeck, Kenneth H., Ellerby, Lisa M., Morrison, Richard S., Taylor, J. Paul, La Spada, Albert R.
Vydáno 2009

Antisense oligonucleotides targeting mutant Ataxin-7 restore visual function in a mouse model of spinocerebellar ataxia type 7

Autor Niu, Chenchen, Prakash, Thazah P., Kim, Aneeza, Quach, John L., Huryn, Laryssa A., Yang, Yuechen, Lopez, Edith, Jazayeri, Ali, Hung, Gene, Sopher, Bryce L., Brooks, Brian P., Swayze, Eric E., Bennett, C. Frank, La Spada, Albert R.
Vydáno v Sci Transl Med (2018)

A Novel Antisense CAG Repeat Transcript at JPH3 Locus Mediating Expanded Polyglutamine Protein Toxicity in Huntington’s Disease-Like 2 (HDL2) Mice

Autor Wilburn, Brian, Rudnicki, Dobrila D., Zhao, Jing, Weitz, Tara Murphy, Cheng, Yin, Gu, Xiaofeng, Greiner, Erin, Park, Chang Sin, Wang, Nan, Sopher, Bryce L., La Spada, Albert R., Osmand, Alex, Margolis, Russell L., Sun, Yi E., Yang, X. William
Vydáno 2011

CTCF cis-Regulates Trinucleotide Repeat Instability in an Epigenetic Manner: A Novel Basis for Mutational Hot Spot Determination

Autor Libby, Randell T., Hagerman, Katharine A., Pineda, Victor V., Lau, Rachel, Cho, Diane H., Baccam, Sandy L., Axford, Michelle M., Cleary, John D., Moore, James M., Sopher, Bryce L., Tapscott, Stephen J., Filippova, Galina N., Pearson, Christopher E., La Spada, Albert R.
Vydáno 2008