Search Results - "Brown, Robert H."

Decoding ALS: from genes to mechanism by Taylor, J. Paul, Brown, Robert H., Cleveland, Don W.

“…Amyotrophic lateral sclerosis (ALS) is a progressive and uniformly fatal neurodegenerative disease. A plethora of genetic factors have been identified that…”
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Emerging mechanisms of molecular pathology in ALS by Peters, Owen M, Ghasemi, Mehdi, Brown, Jr, Robert H

“…Amyotrophic lateral sclerosis (ALS) is a devastating degenerative disease characterized by progressive loss of motor neurons in the motor cortex, brainstem,…”
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Intrinsic membrane hyperexcitability of amyotrophic lateral sclerosis patient-derived motor neurons by Wainger, Brian J, Kiskinis, Evangelos, Mellin, Cassidy, Wiskow, Ole, Han, Steve S W, Sandoe, Jackson, Perez, Numa P, Williams, Luis A, Lee, Seungkyu, Boulting, Gabriella, Berry, James D, Brown, Jr, Robert H, Cudkowicz, Merit E, Bean, Bruce P, Eggan, Kevin, Woolf, Clifford J

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of the motor nervous system. We show using multielectrode array and patch-clamp…”
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Amyotrophic lateral sclerosis: Problems and prospects by Sreedharan, Jemeen, Brown Jr, Robert H.

“…Amyotrophic lateral sclerosis (ALS) is a lethal degenerative disorder of motoneurons, which may occur concurrently with frontotemporal dementia. Genetic…”
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Molecular biology of amyotrophic lateral sclerosis: insights from genetics by Pasinelli, Piera, Brown, Robert H

“…Amyotrophic lateral sclerosis (ALS) is a paralytic disorder caused by motor neuron degeneration. Mutations in more than 50 human genes cause diverse types of…”
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Gene therapy for neurologic disorders by Eichler, Florian S., Brown, Robert H.

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Genetics of Amyotrophic Lateral Sclerosis by Ghasemi, Mehdi, Brown, Jr, Robert H

“…Amyotrophic lateral sclerosis (ALS) is a devastating, uniformly lethal degenerative disorder of motor neurons that overlaps clinically with frontotemporal…”
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Silencing strategies for therapy of SOD1-mediated ALS by van Zundert, Brigitte, Brown, Robert H.

“…Amyotrophic lateral sclerosis (ALS) is an adult-onset, lethal, paralytic disorder caused by the degeneration of motor neurons. Our understanding of this…”
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Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS by Bosco, Daryl A, Brown, Robert H, Morfini, Gerardo, Karabacak, N Murat, Song, Yuyu, Gros-Louis, Francois, Pasinelli, Piera, Goolsby, Holly, Fontaine, Benjamin A, Lemay, Nathan, McKenna-Yasek, Diane, Frosch, Matthew P, Agar, Jeffrey N, Julien, Jean-Pierre, Brady, Scott T

“…Could similar changes in SOD1 underlie both familial and sporadic ALS? Here, Bosco et al . find that wild-type SOD1 from sporadic ALS tissues shows…”
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Suppression of mutant C9orf72 expression by a potent mixed backbone antisense oligonucleotide by Tran, Hélène, Moazami, Michael P., Yang, Huiya, McKenna-Yasek, Diane, Douthwright, Catherine L., Pinto, Courtney, Metterville, Jake, Shin, Minwook, Sanil, Nitasha, Dooley, Craig, Puri, Ajit, Weiss, Alexandra, Wightman, Nicholas, Gray-Edwards, Heather, Marosfoi, Miklos, King, Robert M., Kenderdine, Thomas, Fabris, Daniele, Bowser, Robert, Watts, Jonathan K., Brown, Robert H.

“…Expansions of a G 4 C 2 repeat in the C9ORF72 gene are the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD),…”
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TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD by White, Matthew A., Kim, Eosu, Duffy, Amanda, Adalbert, Robert, Phillips, Benjamin U., Peters, Owen M., Stephenson, Jodie, Yang, Sujeong, Massenzio, Francesca, Lin, Ziqiang, Andrews, Simon, Segonds-Pichon, Anne, Metterville, Jake, Saksida, Lisa M., Mead, Richard, Ribchester, Richard R, Barhomi, Youssef, Serre, Thomas, Coleman, Michael P., Fallon, Justin R., Bussey, Timothy J., Brown, Robert H., Sreedharan, Jemeen

“…Amyotrophic lateral sclerosis–frontotemporal dementia (ALS-FTD) constitutes a devastating disease spectrum characterized by 43-kDa TAR DNA-binding protein…”
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Hereditary Sensory Neuropathy Type 1 Is Caused by the Accumulation of Two Neurotoxic Sphingolipids by Penno, Anke, Reilly, Mary M., Houlden, Henry, Laurá, Matilde, Rentsch, Katharina, Niederkofler, Vera, Stoeckli, Esther T., Nicholson, Garth, Eichler, Florian, Brown, Robert H., von Eckardstein, Arnold, Hornemann, Thorsten

“…HSAN1 is an inherited neuropathy found to be associated with several missense mutations in the SPTLC1 subunit of serine palmitoyltransferase (SPT). SPT…”
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Adeno-associated virus-delivered artificial microRNA extends survival and delays paralysis in an amyotrophic lateral sclerosis mouse model by Stoica, Lorelei, Todeasa, Sophia H., Cabrera, Gabriela Toro, Salameh, Johnny S., ElMallah, Mai K., Mueller, Christian, Brown Jr, Robert H., Sena-Esteves, Miguel

“…Objective Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by loss of motor neurons, resulting in progressive muscle…”
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EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans by Van Hoecke, Annelies, Schoonaert, Lies, Lemmens, Robin, Timmers, Mieke, Staats, Kim A, Laird, Angela S, Peeters, Elke, Philips, Thomas, Goris, An, Dubois, Bénédicte, Andersen, Peter M, Al-Chalabi, Ammar, Thijs, Vincent, Turnley, Ann M, van Vught, Paul W, Veldink, Jan H, Hardiman, Orla, Van Den Bosch, Ludo, Gonzalez-Perez, Paloma, Van Damme, Philip, Brown, Robert H, van den Berg, Leonard H, Robberecht, Wim

“…Epha4 is a receptor involved in axonal repulsion. Wim Robberecht and his colleagues report that genetic or pharmacological inhibition of Epha4 is protective in…”
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Emerging mechanisms of molecular pathology in ALS by Peters, Owen M, Ghasemi, Mehdi, Brown, Jr, Robert H

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Axonal Transport Defects in Neurodegenerative Diseases by Morfini, Gerardo A, Burns, Matthew, Binder, Lester I, Kanaan, Nicholas M, LaPointe, Nichole, Bosco, Daryl A, Brown, Robert H., Jr, Brown, Hannah, Tiwari, Ashutosh, Hayward, Lawrence, Edgar, Julia, Nave, Klaus-Armin, Garberrn, James, Atagi, Yuka, Song, Yuyu, Pigino, Gustavo, Brady, Scott T

“…Adult-onset neurodegenerative diseases (AONDs) comprise a heterogeneous group of neurological disorders characterized by a progressive, age-dependent decline…”
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T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS by Chiu, Isaac M, Chen, Adam, Zheng, Yi, Kosaras, Bela, Tsiftsoglou, Stefanos A, Vartanian, Timothy K, Brown, Robert H. Jr, Carroll, Michael C

“…Amyotrophic Lateral Sclerosis (ALS) is an adult-onset, progressive, motor neuron degenerative disease, in which the role of inflammation is not well…”
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Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis by Yang, Chunxing, Wang, Hongyan, Qiao, Tao, Yang, Bin, Aliaga, Leonardo, Qiu, Linghua, Tan, Weijia, Salameh, Johnny, McKenna-Yasek, Diane M., Smith, Thomas, Peng, Lingtao, Moore, Melissa J., Brown, Robert H., Cai, Huaibin, Xu, Zuoshang

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease that causes motor neuron degeneration, progressive motor dysfunction, paralysis, and death…”
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Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice by Peters, Owen M., Cabrera, Gabriela Toro, Tran, Helene, Gendron, Tania F., McKeon, Jeanne E., Metterville, Jake, Weiss, Alexandra, Wightman, Nicholas, Salameh, Johnny, Kim, Juhyun, Sun, Huaming, Boylan, Kevin B., Dickson, Dennis, Kennedy, Zachary, Lin, Ziqiang, Zhang, Yong-Jie, Daughrity, Lillian, Jung, Chris, Gao, Fen-Biao, Sapp, Peter C., Horvitz, H. Robert, Bosco, Daryl A., Brown, Solange P., de Jong, Pieter, Petrucelli, Leonard, Mueller, Christian, Brown, Robert H.

“…A non-coding hexanucleotide repeat expansion in the C9ORF72 gene is the most common mutation associated with familial amyotrophic lateral sclerosis (ALS) and…”
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dSarm/Sarm1 Is Required for Activation of an Injury-Induced Axon Death Pathway by Osterloh, Jeannette M., Yang, Jing, Rooney, Timothy M., Fox, A. Nicole, Adalbert, Robert, Powell, Eric H., Sheehan, Amy E., Avery, Michelle A., Hackett, Rachel, Logan, Mary A., MacDonald, Jennifer M., Ziegenfuss, Jennifer S., Milde, Stefan, Hou, Ying-Ju, Nathan, Carl, Ding, Aihao, Brown, Robert H., Conforti, Laura, Coleman, Michael, Tessier-Lavigne, Marc, Züchner, Stephan, Freeman, Marc R.

“…Axonal and synaptic degeneration is a hallmark of peripheral neuropathy, brain injury, and neurodegenerative disease. Axonal degeneration has been proposed to…”
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