Search Results - "Riley, Brigit E"

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease by Hipp, Mark S, Patel, Chetan N, Bersuker, Kirill, Riley, Brigit E, Kaiser, Stephen E, Shaler, Thomas A, Brandeis, Michael, Kopito, Ron R

“…Pathognomonic accumulation of ubiquitin (Ub) conjugates in human neurodegenerative diseases, such as Huntington's disease, suggests that highly aggregated…”
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HDAC6 and Microtubules Are Required for Autophagic Degradation of Aggregated Huntingtin by Iwata, Atsushi, Riley, Brigit E., Johnston, Jennifer A., Kopito, Ron R.

“…CNS neurons are endowed with the ability to recover from cytotoxic insults associated with the accumulation of proteinaceous polyglutamine aggregates via a…”
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Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle by Riley, Brigit E, Orr, Harry T

“…The polyglutamine disorders are a class of nine neuro-degenerative disorders that are inherited gain-of-function diseases caused by expansion of a translated…”
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Ubiquitin accumulation in autophagy-deficient mice is dependent on the Nrf2-mediated stress response pathway: a potential role for protein aggregation in autophagic substrate selection by Riley, Brigit E, Kaiser, Stephen E, Shaler, Thomas A, Ng, Aylwin C.Y, Hara, Taichi, Hipp, Mark S, Lage, Kasper, Xavier, Ramnik J, Ryu, Kwon-Yul, Taguchi, Keiko, Yamamoto, Masayuki, Tanaka, Keiji, Mizushima, Noboru, Komatsu, Masaaki, Kopito, Ron R

“…Genetic ablation of autophagy in mice leads to liver and brain degeneration accompanied by the appearance of ubiquitin (Ub) inclusions, which has been…”
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Systems-based analyses of brain regions functionally impacted in Parkinson's disease reveals underlying causal mechanisms by Riley, Brigit E, Gardai, Shyra J, Emig-Agius, Dorothea, Bessarabova, Marina, Ivliev, Alexander E, Schüle, Birgitt, Schüle, Birgit, Alexander, Jeff, Wallace, William, Halliday, Glenda M, Langston, J William, Braxton, Scott, Yednock, Ted, Shaler, Thomas, Johnston, Jennifer A

“…Detailed analysis of disease-affected tissue provides insight into molecular mechanisms contributing to pathogenesis. Substantia nigra, striatum, and cortex…”
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A polyubiquitin chain reaction: parkin recruitment to damaged mitochondria by Riley, Brigit E, Olzmann, James A

“…  [...]the mechanism underlying PINK1-mediated Parkin recruitment remained a mystery. [...]it will be imperative to determine the therapeutic potential of…”
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Autophagy inhibition engages Nrf2-p62 Ub-associated signaling by Riley, Brigit E., Kaiser, Stephen E., Kopito, Ron R.

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SUMOylation of the Polyglutamine Repeat Protein, Ataxin-1, Is Dependent on a Functional Nuclear Localization Signal by Riley, Brigit E., Zoghbi, Huda Y., Orr, Harry T.

“…SUMO (small ubiquitin-like modifier) is a member of the ubiquitin family of proteins. SUMO targets include proteins involved in numerous roles including…”
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Protein standard absolute quantification (PSAQ) method for the measurement of cellular ubiquitin pools by Kaiser, Stephen E, Riley, Brigit E, Shaler, Thomas A, Trevino, R Sean, Becker, Christopher H, Schulman, Howard, Kopito, Ron R

“…Ubiquitin, an important post-translational modification that regulates a variety of biological processes is found in free and conjugated (monoubiquitin and…”
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The Effects of the Polyglutamine Repeat Protein Ataxin-1 on the UbL-UBA Protein A1Up by Riley, Brigit E, Xu, Yifan, Zoghbi, Huda Y, Orr, Harry T

“…The ataxin-1 interacting ubiquitin-like protein (A1Up) contains an amino-terminal ubiquitin-like (UbL) region, four stress-inducible, heat shock…”
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Preclinical evaluation of ADVM-062, a novel intravitreal gene therapy vector for the treatment of blue cone monochromacy by Hanna, Kelly, Nieves, Julio, Dowd, Christine, Bender, Kristina Oresic, Sharma, Pallavi, Singh, Baljit, Renz, Mark, Ver Hoeve, James N., Cepeda, Diana, Gelfman, Claire M., Riley, Brigit E., Grishanin, Ruslan N.

“…Blue cone monochromacy (BCM) is a rare X-linked retinal disease characterized by the absence of L- and M-opsin in cone photoreceptors, considered a potential…”
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Cellular mechanisms of protein quality control by Bennett, Eric J, Shaler, Tom, Gonzalez-Zulueta, Mirella, Schulman, Howard F, Iwata, Atsushi, Riley, Brigit E, Johnston, Jennifer A, Bucci, Mirella, Nukina, Noboyuki, Ellerby, Lisa, Kopito, Ron R

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A Polyubiquitin Chain Reaction: Parkin Recruitment to Damaged Mitochondria: e1004952 by Riley, Brigit E, Olzmann, James A

“…[...]the mechanism underlying PINK1-mediated Parkin recruitment remained a mystery. [...]it will be imperative to determine the therapeutic potential of small…”
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Development of an Optimized rAAV2/6 Human Factor 8 cDNA Vector Cassette for Hemophilia a Gene Therapy by Riley, Brigit E., Boonsripisal, Jeff, Goodwin, Alicia, Cartier, Dominique, Garces, Eudean, Nesbitt, Kate, Dwyer, Courtney, Ballaron, Stephen, Hebert, Kassandra, Tran, Hung, Kang, Andrea, Zhang, Lei, Meyer, Kathleen E., Greengard, Judith, Hough, Christine, Surosky, Richard, Ando, Dale Genji, Lillicrap, David, Nichol, Geoff, Holmes, Michael C.

“…Hemophilia A, which is caused by a mutation in the Factor 8 (F8) gene resulting in a deficiency or lack of the Factor VIII (FVIII) protein, is the most common…”
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