Search Results - "WRAY, Selina"

C9orf72 expansions in frontotemporal dementia and amyotrophic lateral sclerosis by Rohrer, Jonathan D, Dr, Isaacs, Adrian M, PhD, Mizielinska, Sarah, PhD, Mead, Simon, Prof, Lashley, Tammaryn, PhD, Wray, Selina, PhD, Sidle, Katie, PhD, Fratta, Pietro, PhD, Orrell, Richard W, MD, Hardy, John, Prof, Holton, Janice, PhD, Revesz, Tamas, Prof, Rossor, Martin N, Prof, Warren, Jason D, Prof

“…Summary C9orf72 hexanucleotide repeat expansions are the most common cause of familial frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS)…”
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Neuronal activity enhances tau propagation and tau pathology in vivo by Wu, Jessica W, Hussaini, S Abid, Bastille, Isle M, Rodriguez, Gustavo A, Mrejeru, Ana, Rilett, Kelly, Sanders, David W, Cook, Casey, Fu, Hongjun, Boonen, Rick A C M, Herman, Mathieu, Nahmani, Eden, Emrani, Sheina, Figueroa, Y Helen, Diamond, Marc I, Clelland, Catherine L, Wray, Selina, Duff, Karen E

“…The authors show that tau can be released by neurons and transferred to other neurons via the extracellular space. Moreover, they show that enhancing neuronal…”
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Microtubules Deform the Nuclear Membrane and Disrupt Nucleocytoplasmic Transport in Tau-Mediated Frontotemporal Dementia by Paonessa, Francesco, Evans, Lewis D., Solanki, Ravi, Larrieu, Delphine, Wray, Selina, Hardy, John, Jackson, Stephen P., Livesey, Frederick J.

“…The neuronal microtubule-associated protein tau, MAPT, is central to the pathogenesis of many dementias. Autosomal-dominant mutations in MAPT cause inherited…”
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Tau Protein Hyperphosphorylation and Aggregation in Alzheimer's Disease and Other Tauopathies, and Possible Neuroprotective Strategies by Šimić, Goran, Babić Leko, Mirjana, Wray, Selina, Harrington, Charles, Delalle, Ivana, Jovanov-Milošević, Nataša, Bažadona, Danira, Buée, Luc, de Silva, Rohan, Di Giovanni, Giuseppe, Wischik, Claude, Hof, Patrick R

“…Abnormal deposition of misprocessed and aggregated proteins is a common final pathway of most neurodegenerative diseases, including Alzheimer's disease (AD)…”
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The Src/c-Abl pathway is a potential therapeutic target in amyotrophic lateral sclerosis by Imamura, Keiko, Izumi, Yuishin, Watanabe, Akira, Tsukita, Kayoko, Woltjen, Knut, Yamamoto, Takuya, Hotta, Akitsu, Kondo, Takayuki, Kitaoka, Shiho, Ohta, Akira, Tanaka, Akito, Watanabe, Dai, Morita, Mitsuya, Takuma, Hiroshi, Tamaoka, Akira, Kunath, Tilo, Wray, Selina, Furuya, Hirokazu, Era, Takumi, Makioka, Kouki, Okamoto, Koichi, Fujisawa, Takao, Nishitoh, Hideki, Homma, Kengo, Ichijo, Hidenori, Julien, Jean-Pierre, Obata, Nanako, Hosokawa, Masato, Akiyama, Haruhiko, Kaneko, Satoshi, Ayaki, Takashi, Ito, Hidefumi, Kaji, Ryuji, Takahashi, Ryosuke, Yamanaka, Shinya, Inoue, Haruhisa

“…Amyotrophic lateral sclerosis (ALS), a fatal disease causing progressive loss of motor neurons, still has no effective treatment. We developed a phenotypic…”
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Familial Alzheimer’s Disease Mutations in PSEN1 Lead to Premature Human Stem Cell Neurogenesis by Arber, Charles, Lovejoy, Christopher, Harris, Lachlan, Willumsen, Nanet, Alatza, Argyro, Casey, Jackie M., Lines, Georgie, Kerins, Caoimhe, Mueller, Anika K., Zetterberg, Henrik, Hardy, John, Ryan, Natalie S., Fox, Nick C., Lashley, Tammaryn, Wray, Selina

“…Mutations in presenilin 1 (PSEN1) or presenilin 2 (PSEN2), the catalytic subunit of γ-secretase, cause familial Alzheimer’s disease (fAD). We hypothesized that…”
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G‐quadruplex‐binding small molecules ameliorate C9orf72 FTD/ALS pathology in vitro and in vivo by Simone, Roberto, Balendra, Rubika, Moens, Thomas G, Preza, Elisavet, Wilson, Katherine M, Heslegrave, Amanda, Woodling, Nathan S, Niccoli, Teresa, Gilbert‐Jaramillo, Javier, Abdelkarim, Samir, Clayton, Emma L, Clarke, Mica, Konrad, Marie‐Therese, Nicoll, Andrew J, Mitchell, Jamie S, Calvo, Andrea, Chio, Adriano, Houlden, Henry, Polke, James M, Ismail, Mohamed A, Stephens, Chad E, Vo, Tam, Farahat, Abdelbasset A, Wilson, W David, Boykin, David W, Zetterberg, Henrik, Partridge, Linda, Wray, Selina, Parkinson, Gary, Neidle, Stephen, Patani, Rickie, Fratta, Pietro, Isaacs, Adrian M

“…Intronic GGGGCC repeat expansions in C9orf72 are the most common known cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), which…”
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Maturation and phenotype of pathophysiological neuronal excitability of human cells in tau-related dementia by Kopach, Olga, Esteras, Noemí, Wray, Selina, Rusakov, Dmitri A, Abramov, Andrey Y

“…Frontotemporal dementia and parkinsonism (FTDP-17) caused by the 10+16 splice-site mutation in the gene encoding microtubule-associated protein tau ( )…”
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Excess α-synuclein compromises phagocytosis in iPSC-derived macrophages by Haenseler, Walther, Zambon, Federico, Lee, Heyne, Vowles, Jane, Rinaldi, Federica, Duggal, Galbha, Houlden, Henry, Gwinn, Katrina, Wray, Selina, Luk, Kelvin C., Wade-Martins, Richard, James, William S., Cowley, Sally A.

“…To examine the pathogenic role of α-synuclein (αS) in Parkinson’s Disease, we have generated induced Pluripotent Stem Cell lines from early onset Parkinson’s…”
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Mitochondrial hyperpolarization in iPSC-derived neurons from patients of FTDP-17 with 10+16 MAPT mutation leads to oxidative stress and neurodegeneration by Esteras, Noemí, Rohrer, Jonathan D, Hardy, John, Wray, Selina, Abramov, Andrey Y

“…Tau protein inclusions are a frequent hallmark of a variety of neurodegenerative disorders. The 10+16 intronic mutation in MAPT gene, encoding tau, causes…”
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Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS by Hall, Claire E., Yao, Zhi, Choi, Minee, Tyzack, Giulia E., Serio, Andrea, Luisier, Raphaelle, Harley, Jasmine, Preza, Elisavet, Arber, Charlie, Crisp, Sarah J., Watson, P. Marc D., Kullmann, Dimitri M., Abramov, Andrey Y., Wray, Selina, Burley, Russell, Loh, Samantha H.Y., Martins, L. Miguel, Stevens, Molly M., Luscombe, Nicholas M., Sibley, Christopher R., Lakatos, Andras, Ule, Jernej, Gandhi, Sonia, Patani, Rickie

“…Motor neurons (MNs) and astrocytes (ACs) are implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS), but their interaction and the sequence of…”
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MAPT expression and splicing is differentially regulated by brain region: relation to genotype and implication for tauopathies by TRABZUNI, Daniah, WRAY, Selina, AREPALLI, Sampath, SINGLETON, Andrew B, COOKSON, Mark R, PITTMAN, Alan M, DE SILVA, Rohan, WEALE, Michael E, HARDY, John, RYTEN, Mina, VANDROVCOVA, Jana, RAMASAMY, Adaikalavan, WALKER, Robert, SMITH, Colin, LUK, Connie, RAPHAEL GIBBS, J, DILLMAN, Allissa, HERNANDEZ, Dena G

“…The MAPT (microtubule-associated protein tau) locus is one of the most remarkable in neurogenetics due not only to its involvement in multiple…”
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Selective suppression of oligodendrocyte-derived amyloid beta rescues neuronal dysfunction in Alzheimer's disease by Rajani, Rikesh M, Ellingford, Robert, Hellmuth, Mariam, Harris, Samuel S, Taso, Orjona S, Graykowski, David, Lam, Francesca Kar Wey, Arber, Charles, Fertan, Emre, Danial, John S H, Swire, Matthew, Lloyd, Marcus, Giovannucci, Tatiana A, Bourdenx, Mathieu, Klenerman, David, Vassar, Robert, Wray, Selina, Sala Frigerio, Carlo, Busche, Marc Aurel

“…Reduction of amyloid beta (Aβ) has been shown to be effective in treating Alzheimer's disease (AD), but the underlying assumption that neurons are the main…”
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Editorial: Mechanisms of Action in Neurodegenerative Proteinopathies by Miller, Sean J., Wray, Selina, Sattler, Rita, Zhang, Can

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Genetically engineered MAPT 10+16 mutation causes pathophysiological excitability of human iPSC-derived neurons related to 4R tau-induced dementia by Kopach, Olga, Esteras, Noemí, Wray, Selina, Abramov, Andrey Y., Rusakov, Dmitri A.

“…Human iPSC lines represent a powerful translational model of tauopathies. We have recently described a pathophysiological phenotype of neuronal excitability of…”
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Novel Phosphorylation Sites in Tau from Alzheimer Brain Support a Role for Casein Kinase 1 in Disease Pathogenesis by Hanger, Diane P., Byers, Helen L., Wray, Selina, Leung, Kit-Yi, Saxton, Malcolm J., Seereeram, Anjan, Reynolds, C. Hugh, Ward, Malcolm A., Anderton, Brian H.

“…Tau in Alzheimer disease brain is highly phosphorylated and aggregated into paired helical filaments comprising characteristic neurofibrillary tangles. Here we…”
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Knockdown of Amyloid Precursor Protein: Biological Consequences and Clinical Opportunities by Gabriele, Rebecca M C, Abel, Emily, Fox, Nick C, Wray, Selina, Arber, Charles

“…Amyloid precursor protein ( ) and its cleavage fragment Amyloid-β (Aβ) have fundamental roles in Alzheimer's disease (AD). Genetic alterations that either…”
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Pathogenic VCP Mutations Induce Mitochondrial Uncoupling and Reduced ATP Levels by Bartolome, Fernando, Wu, Hsiu-Chuan, Burchell, Victoria S., Preza, Elisavet, Wray, Selina, Mahoney, Colin J., Fox, Nick C., Calvo, Andrea, Canosa, Antonio, Moglia, Cristina, Mandrioli, Jessica, Chiò, Adriano, Orrell, Richard W., Houlden, Henry, Hardy, John, Abramov, Andrey Y., Plun-Favreau, Helene

“…Valosin-containing protein (VCP) is a highly expressed member of the type II AAA+ ATPase family. VCP mutations are the cause of inclusion body myopathy,…”
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iPSC-derived neuronal models of PANK2-associated neurodegeneration reveal mitochondrial dysfunction contributing to early disease by Arber, Charles, Angelova, Plamena R, Wiethoff, Sarah, Tsuchiya, Yugo, Mazzacuva, Francesca, Preza, Elisavet, Bhatia, Kailash P, Mills, Kevin, Gout, Ivan, Abramov, Andrey Y, Hardy, John, Duce, James A, Houlden, Henry, Wray, Selina

“…Mutations in PANK2 lead to neurodegeneration with brain iron accumulation. PANK2 has a role in the biosynthesis of coenzyme A (CoA) from dietary vitamin B5,…”
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Tau cleavage and tau aggregation in neurodegenerative disease by Hanger, Diane P, Wray, Selina

“…Deposition of highly phosphorylated tau in the brain is the most significant neuropathological and biochemical characteristic of the group of neurodegenerative…”
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