Search Results - "McLean, Jesse R."

Glucocerebrosidase gene therapy prevents α-synucleinopathy of midbrain dopamine neurons by Rocha, Emily M, Smith, Gaynor A, Park, Eric, Cao, Hongmei, Brown, Eilish, Hayes, Melissa A, Beagan, Jonathan, McLean, Jesse R, Izen, Sarah C, Perez-Torres, Eduardo, Hallett, Penelope J, Isacson, Ole

“…Abstract Diminished lysosomal function can lead to abnormal cellular accumulation of specific proteins, including α-synuclein, contributing to disease…”
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Alpha-synuclein overexpressing transgenic mice show internal organ pathology and autonomic deficits by Hallett, Penelope J, McLean, Jesse R, Kartunen, Andrew, Langston, J. William, Isacson, Ole

“…Abstract While studying transgenic mice that overexpress human wildtype alpha-synuclein (Thy1-ASO, ASO) for typical brain alpha-synucleinopathy and central…”
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Widespread neuron-specific transgene expression in brain and spinal cord following synapsin promoter-driven AAV9 neonatal intracerebroventricular injection by McLean, Jesse R., Smith, Gaynor A., Rocha, Emily M., Hayes, Melissa A., Beagan, Jonathan A., Hallett, Penelope J., Isacson, Ole

“…•Injection of rAAV9 in neonatal mice exhibits high transduction patterns in the CNS.•Ubiquitous promoter usage and varying tropism limit cell-type-specific…”
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A Nurr1 agonist causes neuroprotection in a Parkinson's disease lesion model primed with the toll-like receptor 3 dsRNA inflammatory stimulant poly(I:C) by Smith, Gaynor A, Rocha, Emily M, Rooney, Thomas, Barneoud, Pascal, McLean, Jesse R, Beagan, Jonathan, Osborn, Teresia, Coimbra, Madeleine, Luo, Yongyi, Hallett, Penelope J, Isacson, Ole

“…Dopaminergic neurons in the substantia nigra pars compacta (SNpc) are characterized by the expression of genes required for dopamine synthesis, handling and…”
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Jump from Pre-mutation to Pathologic Expansion in C9orf72 by Xi, Zhengrui, van Blitterswijk, Marka, Zhang, Ming, McGoldrick, Philip, McLean, Jesse R., Yunusova, Yana, Knock, Erin, Moreno, Danielle, Sato, Christine, McKeever, Paul M., Schneider, Raphael, Keith, Julia, Petrescu, Nicolae, Fraser, Paul, Tartaglia, Maria Carmela, Baker, Matthew C., Graff-Radford, Neill R., Boylan, Kevin B., Dickson, Dennis W., Mackenzie, Ian R., Rademakers, Rosa, Robertson, Janice, Zinman, Lorne, Rogaeva, Ekaterina

“…An expanded G4C2 repeat in C9orf72 represents the most common known genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration…”
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Isoform-specific antibodies reveal distinct subcellular localizations of C9orf72 in amyotrophic lateral sclerosis by Xiao, Shangxi, MacNair, Laura, McGoldrick, Philip, McKeever, Paul M., McLean, Jesse R., Zhang, Ming, Keith, Julia, Zinman, Lorne, Rogaeva, Ekaterina, Robertson, Janice

“…Objective A noncoding hexanucleotide repeat expansion in C9orf72 is the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar…”
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Sustained Systemic Glucocerebrosidase Inhibition Induces Brain α-Synuclein Aggregation, Microglia and Complement C1q Activation in Mice by Rocha, Emily M, Smith, Gaynor A, Park, Eric, Cao, Hongmei, Graham, Anne-Renee, Brown, Eilish, McLean, Jesse R, Hayes, Melissa A, Beagan, Jonathan, Izen, Sarah C, Perez-Torres, Eduardo, Hallett, Penelope J, Isacson, Ole

“…Loss-of-function mutations in GBA1, which cause the autosomal recessive lysosomal storage disease, Gaucher disease (GD), are also a key genetic risk factor for…”
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Pharmacological rescue of mitochondrial deficits in iPSC-derived neural cells from patients with familial Parkinson's disease by Cooper, Oliver, Seo, Hyemyung, Andrabi, Shaida, Guardia-Laguarta, Cristina, Graziotto, John, Sundberg, Maria, McLean, Jesse R, Carrillo-Reid, Luis, Xie, Zhong, Osborn, Teresia, Hargus, Gunnar, Deleidi, Michela, Lawson, Tristan, Bogetofte, Helle, Perez-Torres, Eduardo, Clark, Lorraine, Moskowitz, Carol, Mazzulli, Joseph, Chen, Li, Volpicelli-Daley, Laura, Romero, Norma, Jiang, Houbo, Uitti, Ryan J, Huang, Zhigao, Opala, Grzegorz, Scarffe, Leslie A, Dawson, Valina L, Klein, Christine, Feng, Jian, Ross, Owen A, Trojanowski, John Q, Lee, Virginia M-Y, Marder, Karen, Surmeier, D James, Wszolek, Zbigniew K, Przedborski, Serge, Krainc, Dimitri, Dawson, Ted M, Isacson, Ole

“…Parkinson's disease (PD) is a common neurodegenerative disorder caused by genetic and environmental factors that results in degeneration of the nigrostriatal…”
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Progressive axonal transport and synaptic protein changes correlate with behavioral and neuropathological abnormalities in the heterozygous Q175 KI mouse model of Huntington's disease by Smith, Gaynor A, Rocha, Emily M, McLean, Jesse R, Hayes, Melissa A, Izen, Sarah C, Isacson, Ole, Hallett, Penelope J

“…A long-term goal of modeling Huntington's disease (HD) is to recapitulate the cardinal features of the disease in mice that express both mutant and wild-type…”
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Transcript expression levels of full-length alpha-synuclein and its three alternatively spliced variants in Parkinson's disease brain regions and in a transgenic mouse model of alpha-synuclein overexpression by McLean, Jesse R., Hallett, Penelope J., Cooper, Oliver, Stanley, Michael, Isacson, Ole

“…Alternative splicing is a complex post-transcriptional process that can be regulated by cis-acting elements located within genomic non-coding regions. Recent…”
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Isoform-specific expression and ratio changes accompany oxidant-induced peripherin aggregation in a neuroblastoma cell line by McLean, Jesse R, Robertson, Janice

“…Abstract The type III intermediate filament peripherin is found associated with pathological inclusions present within motor neurons of patients with…”
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A two‐hybrid screen identifies an unconventional role for the intermediate filament peripherin in regulating the subcellular distribution of the SNAP25‐interacting protein, SIP30 by Gentil, Benoit J., McLean, Jesse R., Xiao, Shangxi, Zhao, Beibei, Durham, Heather D., Robertson, Janice

“…Peripherin is a type III intermediate filament protein, the expression of which is associated with the acquisition and maintenance of a terminally…”
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An Aggregate-Inducing Peripherin Isoform Generated through Intron Retention Is Upregulated in Amyotrophic Lateral Sclerosis and Associated with Disease Pathology by Xiao, Shangxi, Tjostheim, Sonja, Sanelli, Teresa, McLean, Jesse R, Horne, Patrick, Fan, Yuxin, Ravits, John, Strong, Michael J, Robertson, Janice

“…The neuronal intermediate filament protein peripherin is a component of ubiquitinated inclusions and of axonal spheroids in amyotrophic lateral sclerosis…”
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ALS-associated peripherin spliced transcripts form distinct protein inclusions that are neuroprotective against oxidative stress by McLean, Jesse R., Smith, Gaynor A., Rocha, Emily M., Osborn, Teresia M., Dib, Samar, Hayes, Melissa A., Beagan, Jonathan A., Brown, Tana B., Lawson, Tristan F.S., Hallett, Penelope J., Robertson, Janice, Isacson, Ole

“…Intracellular proteinaceous inclusions are well-documented hallmarks of the fatal motor neuron disorder amyotrophic lateral sclerosis (ALS). The pathological…”
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Temporal profiles of neuronal degeneration, glial proliferation, and cell death in hNFL(+/+) and NFL(−/−)mice by McLean, Jesse R., Sanelli, Teresa R., Leystra-Lantz, Cheryl, He, Bei Ping, Strong, Michael J.

“…Neurofilament (NF) aggregate formation within motor neurons is a pathological hallmark of both the sporadic and familial forms of amyotrophic lateral sclerosis…”
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Isoform‐specific antibodies reveal distinct subcellular localizations of C 9orf72 in amyotrophic lateral sclerosis by Xiao, Shangxi, MacNair, Laura, McGoldrick, Philip, McKeever, Paul M., McLean, Jesse R., Zhang, Ming, Keith, Julia, Zinman, Lorne, Rogaeva, Ekaterina, Robertson, Janice

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Rab1A and Rab3B gene therapy In the Q175 knock In model of Huntington's disease by Smith, Gaynor A, Mangano, Emily N, Hayes, Melissa, McLean, Jesse R, Beagan, Jonathan, Izen, Sara C, Isacson, Ole, Hallett, Penelope J

“…Regulation of synaptic function, vesicular transport and organelle dynamics is fundamental to the normal function of the neuron. These processes are…”
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Population density regulates Drosophila synaptic morphology in a Fasciclin‐II‐dependent manner by Stewart, Bryan A., McLean, Jesse R.

“…Genetic analysis of the Drosophila larval neuromuscular junction has identified some of the key molecules that regulate synaptic plasticity. Among these…”
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A Nurr1 Agonist Causes Neuroprotection in a Parkinson's Disease Lesion Model Primed with the Toll-Like Receptor 3 dsRNA Inflammatory Stimulant Poly(I:C): e0121072 by Smith, Gaynor A, Rocha, Emily M, Rooney, Thomas, Barneoud, Pascal, McLean, Jesse R, Beagan, Jonathan, Osborn, Teresia, Coimbra, Madeleine, Luo, Yongyi, Hallett, Penelope J

“…Dopaminergic neurons in the substantia nigra pars compacta (SNpc) are characterized by the expression of genes required for dopamine synthesis, handling and…”
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