Search Results - "Todd, Peter K."

Molecular mechanisms underlying nucleotide repeat expansion disorders by Malik, Indranil, Kelley, Chase P., Wang, Eric T., Todd, Peter K.

“…The human genome contains over one million short tandem repeats. Expansion of a subset of these repeat tracts underlies over fifty human disorders, including…”
Get full text

CGG Repeat-Associated Non-AUG Translation Utilizes a Cap-Dependent Scanning Mechanism of Initiation to Produce Toxic Proteins by Kearse, Michael G., Green, Katelyn M., Krans, Amy, Rodriguez, Caitlin M., Linsalata, Alexander E., Goldstrohm, Aaron C., Todd, Peter K.

“…Repeat-associated non-AUG (RAN) translation produces toxic polypeptides from nucleotide repeat expansions in the absence of an AUG start codon and contributes…”
Get full text

RNA-mediated neurodegeneration in repeat expansion disorders by Todd, Peter K., Paulson, Henry L.

“…Most neurodegenerative disorders are thought to result primarily from the accumulation of misfolded proteins, which interfere with protein homeostasis in…”
Get full text

RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response by Green, Katelyn M., Glineburg, M. Rebecca, Kearse, Michael G., Flores, Brittany N., Linsalata, Alexander E., Fedak, Stephen J., Goldstrohm, Aaron C., Barmada, Sami J., Todd, Peter K.

“…Repeat-associated non-AUG (RAN) translation allows for unconventional initiation at disease-causing repeat expansions. As RAN translation contributes to…”
Get full text

Native functions of short tandem repeats by Wright, Shannon E, Todd, Peter K

“…Over a third of the human genome is comprised of repetitive sequences, including more than a million short tandem repeats (STRs). While studies of the…”
Get full text

Repeat-associated non-AUG translation from antisense CCG repeats in fragile X tremor/ataxia syndrome by Krans, Amy, Kearse, Michael G., Todd, Peter K.

“…Objective Repeat‐associated non‐AUG (RAN) translation drives production of toxic proteins from pathogenic repeat sequences in multiple untreatable…”
Get full text

Repeat-associated non-AUG (RAN) translation and other molecular mechanisms in Fragile X Tremor Ataxia Syndrome by Glineburg, M. Rebecca, Todd, Peter K., Charlet-Berguerand, Nicolas, Sellier, Chantal

“…•Fragile X Tremor Ataxia Syndrome (FXTAS) is caused by expanded CGG repeats.•Mechanisms of CGG repeat toxicity include RNA protein titration and RAN…”
Get full text

Non-canonical initiation factors modulate repeat-associated non-AUG translation by Green, Katelyn M, Miller, Shannon L, Malik, Indranil, Todd, Peter K

“…Abstract Repeat-associated non-AUG (RAN) translation of expanded repeat-mutation mRNA produces toxic peptides in neurons of patients suffering from…”
Get full text

Sequestration of DROSHA and DGCR8 by Expanded CGG RNA Repeats Alters MicroRNA Processing in Fragile X-Associated Tremor/Ataxia Syndrome by Sellier, Chantal, Freyermuth, Fernande, Tabet, Ricardos, Tran, Tuan, He, Fang, Ruffenach, Frank, Alunni, Violaine, Moine, Herve, Thibault, Christelle, Page, Adeline, Tassone, Flora, Willemsen, Rob, Disney, Matthew D., Hagerman, Paul J., Todd, Peter K., Charlet-Berguerand, Nicolas

“…Fragile X-associated tremor/ataxia syndrome (FXTAS) is an inherited neurodegenerative disorder caused by the expansion of 55–200 CGG repeats in the 5′ UTR of…”
Get full text

CGG repeats trigger translational frameshifts that generate aggregation-prone chimeric proteins by Wright, Shannon E, Rodriguez, Caitlin M, Monroe, Jeremy, Xing, Jiazheng, Krans, Amy, Flores, Brittany N, Barsur, Venkatesha, Ivanova, Magdalena I, Koutmou, Kristin S, Barmada, Sami J, Todd, Peter K

“…Abstract CGG repeat expansions in the FMR1 5’UTR cause the neurodegenerative disease Fragile X-associated tremor/ataxia syndrome (FXTAS). These repeats form…”
Get full text

What repeat expansion disorders can teach us about the Central Dogma by Wang, Eric T., Freudenreich, Catherine H., Gromak, Natalia, Jain, Ankur, Todd, Peter K., Nagai, Yoshitaka

“…Pathogenic repeat sequences underlie several human disorders, including amyotrophic lateral sclerosis, Huntington’s disease, and myotonic dystrophy. Here, we…”
Get full text

CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome by Todd, Peter K., Oh, Seok Yoon, Krans, Amy, He, Fang, Sellier, Chantal, Frazer, Michelle, Renoux, Abigail J., Chen, Kai-chun, Scaglione, K. Matthew, Basrur, Venkatesha, Elenitoba-Johnson, Kojo, Vonsattel, Jean P., Louis, Elan D., Sutton, Michael A., Taylor, J. Paul, Mills, Ryan E., Charlet-Berguerand, Nicholas, Paulson, Henry L.

“…Fragile X-associated tremor ataxia syndrome (FXTAS) results from a CGG repeat expansion in the 5′ UTR of FMR1. This repeat is thought to elicit toxicity as…”
Get full text

RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome by Oh, Seok Yoon, He, Fang, Krans, Amy, Frazer, Michelle, Taylor, J Paul, Paulson, Henry L, Todd, Peter K

“…Fragile X-associated tremor ataxia syndrome (FXTAS) is a neurodegenerative disorder caused by a CGG trinucleotide repeat expansion in the 5' UTR of the Fragile…”
Get full text

Autophagy and the ubiquitin-proteasome system: Collaborators in neuroprotection by Nedelsky, Natalia B., Todd, Peter K., Taylor, J. Paul

“…Protein degradation is an essential cellular function that, when dysregulated or impaired, can lead to a wide variety of disease states. The two major…”
Get full text

Ribosomal quality control factors inhibit repeat-associated non-AUG translation from GC-rich repeats by Tseng, Yi-Ju, Krans, Amy, Malik, Indranil, Deng, Xiexiong, Yildirim, Evrim, Ovunc, Sinem, Tank, Elizabeth M H, Jansen-West, Karen, Kaufhold, Ross, Gomez, Nicolas B, Sher, Roger, Petrucelli, Leonard, Barmada, Sami J, Todd, Peter K

“…A GGGGCC (G4C2) hexanucleotide repeat expansion in C9ORF72 causes amyotrophic lateral sclerosis and frontotemporal dementia (C9ALS/FTD), while a CGG…”
Get full text

Translation of upstream open reading frames in a model of neuronal differentiation by Rodriguez, Caitlin M, Chun, Sang Y, Mills, Ryan E, Todd, Peter K

“…Upstream open reading frames (uORFs) initiate translation within mRNA 5' leaders, and have the potential to alter main coding sequence (CDS) translation on…”
Get full text

A native function for RAN translation and CGG repeats in regulating fragile X protein synthesis by Rodriguez, Caitlin M., Wright, Shannon E., Kearse, Michael G., Haenfler, Jill M., Flores, Brittany N., Liu, Yu, Ifrim, Marius F., Glineburg, Mary R., Krans, Amy, Jafar-Nejad, Paymaan, Sutton, Michael A., Bassell, Gary J., Parent, Jack M., Rigo, Frank, Barmada, Sami J., Todd, Peter K.

“…Repeat-associated non-AUG-initiated translation of expanded CGG repeats (CGG RAN) from the FMR1 5′-leader produces toxic proteins that contribute to…”
Get full text

Stress granule formation helps to mitigate neurodegeneration by Glineburg, M Rebecca, Yildirim, Evrim, Gomez, Nicolas, Rodriguez, Genesis, Pak, Jaclyn, Li, Xingli, Altheim, Christopher, Waksmacki, Jacob, McInerney, Gerald M, Barmada, Sami J, Todd, Peter K

“…Abstract Cellular stress pathways that inhibit translation initiation lead to transient formation of cytoplasmic RNA/protein complexes known as stress…”
Get full text

Neuropathology of RAN translation proteins in fragile X-associated tremor/ataxia syndrome by Krans, Amy, Skariah, Geena, Zhang, Yuan, Bayly, Bryana, Todd, Peter K

“…CGG repeat expansions in FMR1 cause the neurodegenerative disorder Fragile X-associated Tremor/Ataxia Syndrome (FXTAS). Ubiquitinated neuronal intranuclear…”
Get full text

High-throughput screening yields several small-molecule inhibitors of repeat-associated non-AUG translation by Green, Katelyn M., Sheth, Udit J., Flores, Brittany N., Wright, Shannon E., Sutter, Alexandra B., Kearse, Michael G., Barmada, Sami J., Ivanova, Magdalena I., Todd, Peter K.

“…Repeat-associated non-AUG (RAN) translation is a noncanonical translation initiation event that occurs at nucleotide-repeat expansion mutations that are…”
Get full text