Search Results - "Huang, Eric J."

Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis by Shang, Yulei, Huang, Eric J

“…Abstract Recent advances in the genetics of amyotrophic lateral sclerosis (ALS) have provided key mechanistic insights to the pathogenesis of this devastating…”
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Plastin 3 rescues BDNF-TrkB signaling in spinal muscular atrophy by Ramsey, Arren, Huang, Eric J

“…In this issue, Hennlein and colleagues (2023. J. Cell Biol.https://doi.org/10.1083/jcb.202204113) show that F-actin-bundling protein Plastin 3 is drastically…”
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Secretory autophagy maintains proteostasis upon lysosome inhibition by Solvik, Tina A, Nguyen, Tan A, Tony Lin, Yu-Hsiu, Marsh, Timothy, Huang, Eric J, Wiita, Arun P, Debnath, Jayanta, Leidal, Andrew M

“…The endolysosome system plays central roles in both autophagic degradation and secretory pathways, including the release of extracellular vesicles and…”
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Aldehyde dehydrogenase 1a1 mediates a GABA synthesis pathway in midbrain dopaminergic neurons by Kim, Jae-Ick, Ganesan, Subhashree, Luo, Sarah X, Wu, Yu-Wei, Park, Esther, Huang, Eric J, Chen, Lu, Ding, Jun B

“…Midbrain dopamine neurons are an essential component of the basal ganglia circuitry, playing key roles in the control of fine movement and reward. Recently, it…”
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Comorbid neuropathological diagnoses in early versus late-onset Alzheimer's disease by Spina, Salvatore, La Joie, Renaud, Petersen, Cathrine, Nolan, Amber L, Cuevas, Deion, Cosme, Celica, Hepker, Mackenzie, Hwang, Ji-Hye, Miller, Zachary A, Huang, Eric J, Karydas, Anna M, Grant, Harli, Boxer, Adam L, Gorno-Tempini, Maria Luisa, Rosen, Howard J, Kramer, Joel H, Miller, Bruce L, Seeley, William W, Rabinovici, Gil D, Grinberg, Lea T

“…Copathologies play an important role in the expression of the AD clinical phenotype and may influence treatment efficacy. Early-onset AD (EOAD), defined as…”
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Typical and atypical pathology in primary progressive aphasia variants by Spinelli, Edoardo G., Mandelli, Maria Luisa, Miller, Zachary A., Santos‐Santos, Miguel A, Wilson, Stephen M., Agosta, Federica, Grinberg, Lea T., Huang, Eric J., Trojanowski, John Q., Meyer, Marita, Henry, Maya L., Comi, Giancarlo, Rabinovici, Gil, Rosen, Howard J., Filippi, Massimo, Miller, Bruce L., Seeley, William W., Gorno‐Tempini, Maria Luisa

“…Objective To characterize in vivo signatures of pathological diagnosis in a large cohort of patients with primary progressive aphasia (PPA) variants defined by…”
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Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons by Wang, Wen-Yuan, Pan, Ling, Su, Susan C, Quinn, Emma J, Sasaki, Megumi, Jimenez, Jessica C, Mackenzie, Ian R A, Huang, Eric J, Tsai, Li-Huei

“…Fused-in-Sarcoma ( FUS ) gene encodes an RNA/DNA binding protein whose mutations are linked to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar…”
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Progranulin Deficiency Promotes Circuit-Specific Synaptic Pruning by Microglia via Complement Activation by Lui, Hansen, Zhang, Jiasheng, Makinson, Stefanie R., Cahill, Michelle K., Kelley, Kevin W., Huang, Hsin-Yi, Shang, Yulei, Oldham, Michael C., Martens, Lauren Herl, Gao, Fuying, Coppola, Giovanni, Sloan, Steven A., Hsieh, Christine L., Kim, Charles C., Bigio, Eileen H., Weintraub, Sandra, Mesulam, Marek-Marsel, Rademakers, Rosa, Mackenzie, Ian R., Seeley, William W., Karydas, Anna, Miller, Bruce L., Borroni, Barbara, Ghidoni, Roberta, Farese, Robert V., Paz, Jeanne T., Barres, Ben A., Huang, Eric J.

“…Microglia maintain homeostasis in the brain, but whether aberrant microglial activation can cause neurodegeneration remains controversial. Here, we use…”
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Acetylation of Tau Inhibits Its Degradation and Contributes to Tauopathy by Min, Sang-Won, Cho, Seo-Hyun, Zhou, Yungui, Schroeder, Sebastian, Haroutunian, Vahram, Seeley, William W., Huang, Eric J., Shen, Yong, Masliah, Eliezer, Mukherjee, Chandrani, Meyers, David, Cole, Philip A., Ott, Melanie, Gan, Li

“…Neurodegenerative tauopathies characterized by hyperphosphorylated tau include frontotemporal dementia and Parkinsonism linked to chromosome 17 (FTDP-17) and…”
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Existing Pittsburgh Compound-B positron emission tomography thresholds are too high: statistical and pathological evaluation by Villeneuve, Sylvia, Rabinovici, Gil D, Cohn-Sheehy, Brendan I, Madison, Cindee, Ayakta, Nagehan, Ghosh, Pia M, La Joie, Renaud, Arthur-Bentil, Samia Kate, Vogel, Jacob W, Marks, Shawn M, Lehmann, Manja, Rosen, Howard J, Reed, Bruce, Olichney, John, Boxer, Adam L, Miller, Bruce L, Borys, Ewa, Jin, Lee-Way, Huang, Eric J, Grinberg, Lea T, DeCarli, Charles, Seeley, William W, Jagust, William

“…Amyloid-β, a hallmark of Alzheimer's disease, begins accumulating up to two decades before the onset of dementia, and can be detected in vivo applying…”
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Loss of mitochondrial fission depletes axonal mitochondria in midbrain dopamine neurons by Berthet, Amandine, Margolis, Elyssa B, Zhang, Jue, Hsieh, Ivy, Zhang, Jiasheng, Hnasko, Thomas S, Ahmad, Jawad, Edwards, Robert H, Sesaki, Hiromi, Huang, Eric J, Nakamura, Ken

“…Disruptions in mitochondrial dynamics may contribute to the selective degeneration of dopamine (DA) neurons in Parkinson's disease (PD). However, little is…”
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Self-Organized Cerebral Organoids with Human-Specific Features Predict Effective Drugs to Combat Zika Virus Infection by Watanabe, Momoko, Buth, Jessie E., Vishlaghi, Neda, de la Torre-Ubieta, Luis, Taxidis, Jiannis, Khakh, Baljit S., Coppola, Giovanni, Pearson, Caroline A., Yamauchi, Ken, Gong, Danyang, Dai, Xinghong, Damoiseaux, Robert, Aliyari, Roghiyh, Liebscher, Simone, Schenke-Layland, Katja, Caneda, Christine, Huang, Eric J., Zhang, Ye, Cheng, Genhong, Geschwind, Daniel H., Golshani, Peyman, Sun, Ren, Novitch, Bennett G.

“…The human cerebral cortex possesses distinct structural and functional features that are not found in the lower species traditionally used to model brain…”
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A dramatic increase of C1q protein in the CNS during normal aging by Stephan, Alexander H, Madison, Daniel V, Mateos, José María, Fraser, Deborah A, Lovelett, Emilie A, Coutellier, Laurence, Kim, Leo, Tsai, Hui-Hsin, Huang, Eric J, Rowitch, David H, Berns, Dominic S, Tenner, Andrea J, Shamloo, Mehrdad, Barres, Ben A

“…The decline of cognitive function has emerged as one of the greatest health threats of old age. Age-related cognitive decline is caused by an impacted neuronal…”
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Immature excitatory neurons develop during adolescence in the human amygdala by Sorrells, Shawn F., Paredes, Mercedes F., Velmeshev, Dmitry, Herranz-Pérez, Vicente, Sandoval, Kadellyn, Mayer, Simone, Chang, Edward F., Insausti, Ricardo, Kriegstein, Arnold R., Rubenstein, John L., Manuel Garcia-Verdugo, Jose, Huang, Eric J., Alvarez-Buylla, Arturo

“…The human amygdala grows during childhood, and its abnormal development is linked to mood disorders. The primate amygdala contains a large population of…”
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Neurotoxic microglia promote TDP-43 proteinopathy in progranulin deficiency by Zhang, Jiasheng, Velmeshev, Dmitry, Hashimoto, Kei, Huang, Yu-Hsin, Hofmann, Jeffrey W., Shi, Xiaoyu, Chen, Jiapei, Leidal, Andrew M., Dishart, Julian G., Cahill, Michelle K., Kelley, Kevin W., Liddelow, Shane A., Seeley, William W., Miller, Bruce L., Walther, Tobias C., Farese, Robert V., Taylor, J. Paul, Ullian, Erik M., Huang, Bo, Debnath, Jayanta, Wittmann, Torsten, Kriegstein, Arnold R., Huang, Eric J.

“…Aberrant aggregation of the RNA-binding protein TDP-43 in neurons is a hallmark of frontotemporal lobar degeneration caused by haploinsufficiency in the gene…”
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RNA Binding Proteins and the Pathogenesis of Frontotemporal Lobar Degeneration by Hofmann, Jeffrey W, Seeley, William W, Huang, Eric J

“…Frontotemporal dementia is a group of early onset dementia syndromes linked to underlying frontotemporal lobar degeneration (FTLD) pathology that can be…”
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ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects by Qiu, Haiyan, Lee, Sebum, Shang, Yulei, Wang, Wen-Yuan, Au, Kin Fai, Kamiya, Sherry, Barmada, Sami J, Finkbeiner, Steven, Lui, Hansen, Carlton, Caitlin E, Tang, Amy A, Oldham, Michael C, Wang, Hejia, Shorter, James, Filiano, Anthony J, Roberson, Erik D, Tourtellotte, Warren G, Chen, Bin, Tsai, Li-Huei, Huang, Eric J

“…Autosomal dominant mutations of the RNA/DNA binding protein FUS are linked to familial amyotrophic lateral sclerosis (FALS); however, it is not clear how FUS…”
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CCR2 deficiency impairs macrophage infiltration and improves cognitive function after traumatic brain injury by Hsieh, Christine L, Niemi, Erene C, Wang, Sarah H, Lee, Chih Cheng, Bingham, Deborah, Zhang, Jiasheng, Cozen, Myrna L, Charo, Israel, Huang, Eric J, Liu, Jialing, Nakamura, Mary C

“…Traumatic brain injury (TBI) provokes inflammatory responses, including a dramatic rise in brain macrophages in the area of injury. The pathway(s) responsible…”
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Axin2 as regulatory and therapeutic target in newborn brain injury and remyelination by Rowitch, David H, Fancy, Stephen P J, Harrington, Emily P, Yuen, Tracy J, Silbereis, John C, Zhao, Chao, Baranzini, Sergio E, Bruce, Charlotte C, Otero, Jose J, Huang, Eric J, Nusse, Roel, Franklin, Robin J M

“…Premyelinating oligodendrocytes are vulnerable to hypoxic injuries, especially during the neonatal period. Here, Fancy et al . find that the Wnt scaffolding…”
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Neurons selectively targeted in frontotemporal dementia reveal early stage TDP-43 pathobiology by Nana, Alissa L., Sidhu, Manu, Gaus, Stephanie E., Hwang, Ji-Hye L., Li, Libo, Park, Youngsoon, Kim, Eun-Joo, Pasquini, Lorenzo, Allen, Isabel E., Rankin, Katherine P., Toller, Gianina, Kramer, Joel H., Geschwind, Daniel H., Coppola, Giovanni, Huang, Eric J., Grinberg, Lea T., Miller, Bruce L., Seeley, William W.

“…TAR DNA-binding protein 43 (TDP-43) aggregation is the most common pathological hallmark in frontotemporal dementia (FTD) and characterizes nearly all patients…”
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