Search Results - "Miller, Timothy M."

Distinct Tau Prion Strains Propagate in Cells and Mice and Define Different Tauopathies by Sanders, David W., Kaufman, Sarah K., DeVos, Sarah L., Sharma, Apurwa M., Mirbaha, Hilda, Li, Aimin, Barker, Scarlett J., Foley, Alex C., Thorpe, Julian R., Serpell, Louise C., Miller, Timothy M., Grinberg, Lea T., Seeley, William W., Diamond, Marc I.

“…Prion-like propagation of tau aggregation might underlie the stereotyped progression of neurodegenerative tauopathies. True prions stably maintain unique…”
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Tau Prion Strains Dictate Patterns of Cell Pathology, Progression Rate, and Regional Vulnerability In Vivo by Kaufman, Sarah K., Sanders, David W., Thomas, Talitha L., Ruchinskas, Allison J., Vaquer-Alicea, Jaime, Sharma, Apurwa M., Miller, Timothy M., Diamond, Marc I.

“…Tauopathies are neurodegenerative disorders that affect distinct brain regions, progress at different rates, and exhibit specific patterns of tau accumulation…”
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Emerging antisense oligonucleotide and viral therapies for amyotrophic lateral sclerosis by Ly, Cindy V, Miller, Timothy M

“…Amyotrophic lateral sclerosis (ALS) is a rapidly fatal disease for which there is currently no effective therapy. The present review describes the current…”
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TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration by Wegorzewska, Iga, Bell, Shaughn, Cairns, Nigel J, Miller, Timothy M, Baloh, Robert H

“…Frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) are neurodegenerative diseases that show considerable clinical and pathologic…”
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Increased 4R-Tau Induces Pathological Changes in a Human-Tau Mouse Model by Schoch, Kathleen M., DeVos, Sarah L., Miller, Rebecca L., Chun, Seung J., Norrbom, Michaela, Wozniak, David F., Dawson, Hana N., Bennett, C. Frank, Rigo, Frank, Miller, Timothy M.

“…Pathological evidence for selective four-repeat (4R) tau deposition in certain dementias and exon 10-positioned MAPT mutations together suggest a 4R-specific…”
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RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia by Zu, Tao, Liu, Yuanjing, Bañez-Coronel, Monica, Reid, Tammy, Pletnikova, Olga, Lewis, Jada, Miller, Timothy M, Harms, Matthew B, Falchook, Annet E, Subramony, S H, Ostrow, Lyle W, Rothstein, Jeffrey D, Troncoso, Juan C, Ranum, Laura P W

“…The finding that a GGGGCC (G ₄C ₂) hexanucleotide repeat expansion in the chromosome 9 ORF 72 (C9ORF72) gene is a common cause of amyotrophic lateral sclerosis…”
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Heparan sulfate proteoglycans mediate internalization and propagation of specific proteopathic seeds by Holmes, Brandon B, DeVos, Sarah L, Kfoury, Najla, Li, Mei, Jacks, Rachel, Yanamandra, Kiran, Ouidja, Mohand O, Brodsky, Frances M, Marasa, Jayne, Bagchi, Devika P, Kotzbauer, Paul T, Miller, Timothy M, Papy-Garcia, Dulce, Diamond, Marc I

“…Recent experimental evidence suggests that transcellular propagation of fibrillar protein aggregates drives the progression of neurodegenerative diseases in a…”
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Amyotrophic lateral sclerosis onset is influenced by the burden of rare variants in known amyotrophic lateral sclerosis genes by Cady, Janet, Allred, Peggy, Bali, Taha, Pestronk, Alan, Goate, Alison, Miller, Timothy M., Mitra, Robi D., Ravits, John, Harms, Matthew B., Baloh, Robert H.

“…Objective To define the genetic landscape of amyotrophic lateral sclerosis (ALS) and assess the contribution of possible oligogenic inheritance, we aimed to…”
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Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation by Kanekura, Kohsuke, Yagi, Takuya, Cammack, Alexander J, Mahadevan, Jana, Kuroda, Masahiko, Harms, Matthew B, Miller, Timothy M, Urano, Fumihiko

“…The expansion of the GGGGCC hexanucleotide repeat in the non-coding region of the Chromosome 9 open-reading frame 72 (C9orf72) gene is the most common genetic…”
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Specific RNA interactions promote TDP-43 multivalent phase separation and maintain liquid properties by Grese, Zachary R, Bastos, Alliny CS, Mamede, Lohany D, French, Rachel L, Miller, Timothy M, Ayala, Yuna M

“…TDP-43 is an RNA-binding protein that forms ribonucleoprotein condensates via liquid-liquid phase separation (LLPS) and regulates gene expression through…”
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Method for widespread microRNA-155 inhibition prolongs survival in ALS-model mice by Koval, Erica D, Shaner, Carey, Zhang, Peter, du Maine, Xavier, Fischer, Kimberlee, Tay, Jia, Chau, B Nelson, Wu, Gregory F, Miller, Timothy M

“…microRNAs (miRNAs) are dysregulated in a variety of disease states, suggesting that this newly discovered class of gene expression repressors may be viable…”
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An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study by Miller, Timothy M, Dr, Pestronk, Alan, Prof, David, William, MD, Rothstein, Jeffrey, Prof, Simpson, Ericka, MD, Appel, Stanley H, Prof, Andres, Patricia L, MS, Mahoney, Katy, BA, Allred, Peggy, DPT, Alexander, Katie, BA, Ostrow, Lyle W, MD, Schoenfeld, David, Prof, Macklin, Eric A, PhD, Norris, Daniel A, PhD, Manousakis, Georgios, MD, Crisp, Matthew, BS, Smith, Richard, MD, Bennett, C Frank, PhD, Bishop, Kathie M, PhD, Cudkowicz, Merit E, Prof

“…Summary Background Mutations in SOD1 cause 13% of familial amyotrophic lateral sclerosis. In the SOD1 Gly93Ala rat model of amyotrophic lateral sclerosis, the…”
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CNS microRNA profiles: a database for cell type enriched microRNA expression across the mouse central nervous system by Pomper, Nathan, Liu, Yating, Hoye, Mariah L., Dougherty, Joseph D., Miller, Timothy M.

“…microRNAs are short, noncoding RNAs that can regulate hundreds of targets and thus shape the expression landscape of a cell. Similar to mRNA, they often…”
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Targeted ASO-mediated Atp1a2 knockdown in astrocytes reduces SOD1 aggregation and accelerates disease onset in mutant SOD1 mice by Iyer, Abhirami K, Schoch, Kathleen M, Verbeck, Anthony, Galasso, Grant, Chen, Hao, Smith, Sarah, Oldenborg, Anna, Miller, Timothy M, Karch, Celeste M, Bonni, Azad

“…Astrocyte-specific ion pump α2-Na + /K + -ATPase plays a critical role in the pathogenesis of amyotrophic lateral sclerosis (ALS). Here, we test the effect of…”
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Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria by Vande Velde, Christine, Miller, Timothy M, Cashman, Neil R, Cleveland, Don W

“…Mutations in copper/zinc superoxide dismutase (SOD1) are causative for dominantly inherited amyotrophic lateral sclerosis (ALS). Despite high variability in…”
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Amyotrophic lateral sclerosis care and research in the United States during the COVID‐19 pandemic: Challenges and opportunities by Andrews, Jinsy A., Berry, James D., Baloh, Robert H., Carberry, Nathan, Cudkowicz, Merit E., Dedi, Brixhilda, Glass, Jonathan, Maragakis, Nicholas J., Miller, Timothy M., Paganoni, Sabrina, Rothstein, Jeffrey D., Shefner, Jeremy M., Simmons, Zachary, Weiss, Michael D., Bedlack, Richard S.

“…Coronavirus disease 2019 has created unprecedented challenges for amyotrophic lateral sclerosis (ALS) clinical care and research in the United States…”
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Poly(GR) and poly(GA) in cerebrospinal fluid as potential biomarkers for C9ORF72-ALS/FTD by Krishnan, Gopinath, Raitcheva, Denitza, Bartlett, Daniel, Prudencio, Mercedes, McKenna-Yasek, Diane M., Douthwright, Catherine, Oskarsson, Björn E., Ladha, Shafeeq, King, Oliver D., Barmada, Sami J., Miller, Timothy M., Bowser, Robert, Watts, Jonathan K., Petrucelli, Leonard, Brown, Robert H., Kankel, Mark W., Gao, Fen-Biao

“…GGGGCC repeat expansion in C9ORF72 , which can be translated in both sense and antisense directions into five dipeptide repeat (DPR) proteins, including…”
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Evaluating the efficacy of purchased antisense oligonucleotides to reduce mouse and human tau in vivo by Vemula, Pranav, Schoch, Kathleen M, Miller, Timothy M

“…Many preclinical and clinical studies support the use of antisense oligonucleotides (ASOs) as effective therapeutic strategies. However, acquiring ASOs for…”
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Differential diagnosis of myotonic disorders by Miller, Timothy M.

“…The presence of myotonia and paramyotonia on clinical examination and of myotonic discharges during electrodiagnostic (EDX) studies are important for the…”
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Unraveling the mechanisms involved in motor neuron degeneration in ALS by BRUIJN, Lucie I, MILLER, Timothy M, CLEVELAND, Don W

“…Although Charcot described amyotrophic lateral sclerosis (ALS) more than 130 years ago, the mechanism underlying the characteristic selective degeneration and…”
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