Search Results - "Gitler, D."

Defects in trafficking bridge Parkinson's disease pathology and genetics by Abeliovich, Asa, Gitler, Aaron D.

“…Parkinson's disease is a debilitating, age-associated movement disorder. A central aspect of the pathophysiology of Parkinson's disease is the progressive…”
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Neurodegenerative disease: models, mechanisms, and a new hope by Gitler, Aaron D, Dhillon, Paraminder, Shorter, James

“…Neurodegeneration is a feature of many debilitating, incurable diseases that are rapidly rising in prevalence, such as Parkinson's disease. There is an urgent…”
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Distinct repertoires of microRNAs present in mouse astrocytes compared to astrocyte-secreted exosomes by Jovičić, Ana, Gitler, Aaron D

“…Astrocytes are the most abundant cell type in the central nervous system (CNS) and secrete various factors that regulate neuron development, function and…”
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Stress granules as crucibles of ALS pathogenesis by Li, Yun R, King, Oliver D, Shorter, James, Gitler, Aaron D

“…Amyotrophic lateral sclerosis (ALS) is a fatal human neurodegenerative disease affecting primarily motor neurons. Two RNA-binding proteins, TDP-43 and FUS,…”
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Internalization, axonal transport and release of fibrillar forms of alpha-synuclein by Bieri, Gregor, Gitler, Aaron D, Brahic, Michel

“…Abstract Intra-neuronal protein aggregates made of fibrillar alpha-synuclein (α-syn) are the hallmark of Parkinson's disease (PD). With time, these aggregates…”
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Neurotoxic reactive astrocytes induce cell death via saturated lipids by Guttenplan, Kevin A., Weigel, Maya K., Prakash, Priya, Wijewardhane, Prageeth R., Hasel, Philip, Rufen-Blanchette, Uriel, Münch, Alexandra E., Blum, Jacob A., Fine, Jonathan, Neal, Mikaela C., Bruce, Kimberley D., Gitler, Aaron D., Chopra, Gaurav, Liddelow, Shane A., Barres, Ben A.

“…Astrocytes regulate the response of the central nervous system to disease and injury and have been hypothesized to actively kill neurons in neurodegenerative…”
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Knockout of reactive astrocyte activating factors slows disease progression in an ALS mouse model by Guttenplan, Kevin A., Weigel, Maya K., Adler, Drew I., Couthouis, Julien, Liddelow, Shane A., Gitler, Aaron D., Barres, Ben A.

“…Reactive astrocytes have been implicated in the pathogenesis of neurodegenerative diseases, including a non-cell autonomous effect on motor neuron survival in…”
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Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS by Sun, Zhihui, Diaz, Zamia, Fang, Xiaodong, Hart, Michael P, Chesi, Alessandra, Shorter, James, Gitler, Aaron D

“…TDP-43 and FUS are RNA-binding proteins that form cytoplasmic inclusions in some forms of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar…”
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Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice by Becker, Lindsay A., Huang, Brenda, Bieri, Gregor, Ma, Rosanna, Knowles, David A., Jafar-Nejad, Paymaan, Messing, James, Kim, Hong Joo, Soriano, Armand, Auburger, Georg, Pulst, Stefan M., Taylor, J. Paul, Rigo, Frank, Gitler, Aaron D.

“…A decrease in ataxin-2 levels leads to a reduction in the aggregation of TDP-43, markedly increased lifespan and improved motor function in a transgenic mouse…”
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Single-cell transcriptomic analysis of the adult mouse spinal cord reveals molecular diversity of autonomic and skeletal motor neurons by Blum, Jacob A., Klemm, Sandy, Shadrach, Jennifer L., Guttenplan, Kevin A., Nakayama, Lisa, Kathiria, Arwa, Hoang, Phuong T., Gautier, Olivia, Kaltschmidt, Julia A., Greenleaf, William J., Gitler, Aaron D.

“…The spinal cord is a fascinating structure that is responsible for coordinating movement in vertebrates. Spinal motor neurons control muscle activity by…”
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Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS by Jovičić, Ana, Mertens, Jerome, Boeynaems, Steven, Bogaert, Elke, Chai, Noori, Yamada, Shizuka B, Paul, Joseph W, Sun, Shuying, Herdy, Joseph R, Bieri, Gregor, Kramer, Nicholas J, Gage, Fred H, Van Den Bosch, Ludo, Robberecht, Wim, Gitler, Aaron D

“…C9orf72 mutations are the most common cause of amyotrophic lateral sclerosis and frontotemporal dementia. With unbiased screens in Saccharomyces cerevisiae ,…”
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A prion-like protein regulator of seed germination undergoes hydration-dependent phase separation by Dorone, Yanniv, Boeynaems, Steven, Flores, Eduardo, Jin, Benjamin, Hateley, Shannon, Bossi, Flavia, Lazarus, Elena, Pennington, Janice G., Michiels, Emiel, De Decker, Mathias, Vints, Katlijn, Baatsen, Pieter, Bassel, George W., Otegui, Marisa S., Holehouse, Alex S., Exposito-Alonso, Moises, Sukenik, Shahar, Gitler, Aaron D., Rhee, Seung Y.

“…Many organisms evolved strategies to survive desiccation. Plant seeds protect dehydrated embryos from various stressors and can lay dormant for millennia…”
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CRISPR–Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity by Kramer, Nicholas J., Haney, Michael S., Morgens, David W., Jovičić, Ana, Couthouis, Julien, Li, Amy, Ousey, James, Ma, Rosanna, Bieri, Gregor, Tsui, C. Kimberly, Shi, Yingxiao, Hertz, Nicholas T., Tessier-Lavigne, Marc, Ichida, Justin K., Bassik, Michael C., Gitler, Aaron D.

“…Hexanucleotide-repeat expansions in the C9ORF72 gene are the most common cause of amyotrophic lateral sclerosis and frontotemporal dementia (c9ALS/FTD). The…”
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Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis by Zhang, Yong-Jie, Gendron, Tania F., Ebbert, Mark T. W., O’Raw, Aliesha D., Yue, Mei, Jansen-West, Karen, Zhang, Xu, Prudencio, Mercedes, Chew, Jeannie, Cook, Casey N., Daughrity, Lillian M., Tong, Jimei, Song, Yuping, Pickles, Sarah R., Castanedes-Casey, Monica, Kurti, Aishe, Rademakers, Rosa, Oskarsson, Bjorn, Dickson, Dennis W., Hu, Wenqian, Gitler, Aaron D., Fryer, John D., Petrucelli, Leonard

“…The major genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) is a C9orf72 G 4 C 2 repeat expansion 1 , 2 . Proposed…”
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The material properties of a bacterial-derived biomolecular condensate tune biological function in natural and synthetic systems by Lasker, Keren, Boeynaems, Steven, Lam, Vinson, Scholl, Daniel, Stainton, Emma, Briner, Adam, Jacquemyn, Maarten, Daelemans, Dirk, Deniz, Ashok, Villa, Elizabeth, Holehouse, Alex S., Gitler, Aaron D., Shapiro, Lucy

“…Intracellular phase separation is emerging as a universal principle for organizing biochemical reactions in time and space. It remains incompletely resolved…”
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suite of Gateway® cloning vectors for high-throughput genetic analysis in Saccharomyces cerevisiae by Alberti, Simon, Gitler, Aaron D, Lindquist, Susan

“…In the post-genomic era, academic and biotechnological research is increasingly shifting its attention from single proteins to the analysis of complex protein…”
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A Cellular System that Degrades Misfolded Proteins and Protects against Neurodegeneration by Guo, Lili, Giasson, Benoit I., Glavis-Bloom, Alex, Brewer, Michael D., Shorter, James, Gitler, Aaron D., Yang, Xiaolu

“…Misfolded proteins compromise cellular function and cause disease. How these proteins are detected and degraded is not well understood. Here we show that…”
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ALS-associated ataxin 2 polyQ expansions enhance stress-induced caspase 3 activation and increase TDP-43 pathological modifications by Hart, Michael P, Gitler, Aaron D

“…Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease caused by the loss of motor neurons. The degenerating motor neurons of ALS…”
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TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity by Johnson, Brian S., Snead, David, Lee, Jonathan J., McCaffery, J. Michael, Shorter, James, Gitler, Aaron D.

“…Non-amyloid, ubiquitinated cytoplasmic inclusions containing TDP-43 and its C-terminal fragments are pathological hallmarks of amyotrophic lateral sclerosis…”
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Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD by Boeynaems, Steven, Bogaert, Elke, Michiels, Emiel, Gijselinck, Ilse, Sieben, Anne, Jovičić, Ana, De Baets, Greet, Scheveneels, Wendy, Steyaert, Jolien, Cuijt, Ivy, Verstrepen, Kevin J., Callaerts, Patrick, Rousseau, Frederic, Schymkowitz, Joost, Cruts, Marc, Van Broeckhoven, Christine, Van Damme, Philip, Gitler, Aaron D., Robberecht, Wim, Van Den Bosch, Ludo

“…Hexanucleotide repeat expansions in C9orf72 are the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal degeneration (FTD) (c9ALS/FTD)…”
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