Search Results - "Mazzulli, Joseph"

Molecular mechanisms of α-synuclein and GBA1 in Parkinson’s disease by Stojkovska, Iva, Krainc, Dimitri, Mazzulli, Joseph R.

“…Parkinson’s disease (PD) is a neurodegenerative movement disorder characterized pathologically by the presence of Lewy bodies comprised of insoluble alpha…”
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α-Synuclein–induced lysosomal dysfunction occurs through disruptions in protein trafficking in human midbrain synucleinopathy models by Mazzulli, Joseph R., Zunke, Friederike, Isacson, Ole, Studer, Lorenz, Krainc, Dimitri

“…Parkinson’s disease (PD) is an age-related neurodegenerative disorder characterized by the accumulation of protein aggregates comprised of α-synuclein (α-syn)…”
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Is Parkinson's disease a lysosomal disorder? by Klein, Andrés D, Mazzulli, Joseph R

“…Parkinson's disease has long been considered idiopathic, with most cases having no apparent genetic cause. However, Klein and Mazzulli argue that recent data…”
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Gaucher Disease Glucocerebrosidase and α-Synuclein Form a Bidirectional Pathogenic Loop in Synucleinopathies by Mazzulli, Joseph R., Xu, You-Hai, Sun, Ying, Knight, Adam L., McLean, Pamela J., Caldwell, Guy A., Sidransky, Ellen, Grabowski, Gregory A., Krainc, Dimitri

“…Parkinson's disease (PD), an adult neurodegenerative disorder, has been clinically linked to the lysosomal storage disorder Gaucher disease (GD), but the…”
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Neuroinflammation in Gaucher disease, neuronal ceroid lipofuscinosis, and commonalities with Parkinson’s disease by Francelle, Laetitia, Mazzulli, Joseph R.

“…•Distinct molecular subtypes of microglia and astrocytes exist in the brain that have deleterious or beneficial effects on neurons.•Neuroinflammatory processes…”
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Dopamine oxidation mediates mitochondrial and lysosomal dysfunction in Parkinson’s disease by Burbulla, Lena F., Song, Pingping, Mazzulli, Joseph R., Zampese, Enrico, Wong, Yvette C., Jeon, Sohee, Santos, David P., Blanz, Judith, Obermaier, Carolin D., Strojny, Chelsee, Savas, Jeffrey N., Kiskinis, Evangelos, Zhuang, Xiaoxi, Krüger, Rejko, Surmeier, D. James, Krainc, Dimitri

“…Mitochondrial and lysosomal dysfunction have been implicated in substantia nigra dopaminergic neurodegeneration in Parkinson’s disease (PD), but how these…”
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Dysregulation of the autophagic-lysosomal pathway in Gaucher and Parkinson's disease by Pitcairn, Caleb, Wani, Willayat Yousuf, Mazzulli, Joseph R.

“…The finding that mutations in the Gaucher's Disease (GD) gene GBA1 are a strong risk factor for Parkinson's Disease (PD) has allowed for unique insights into…”
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Reversible Conformational Conversion of α-Synuclein into Toxic Assemblies by Glucosylceramide by Zunke, Friederike, Moise, Alexandra C., Belur, Nandkishore R., Gelyana, Eilrayna, Stojkovska, Iva, Dzaferbegovic, Haris, Toker, Nicholas J., Jeon, Sohee, Fredriksen, Kristina, Mazzulli, Joseph R.

“…α-Synuclein (α-syn) aggregation is a key event in Parkinson’s disease (PD). Mutations in glycosphingolipid (GSL)-degrading glucocerebrosidase are risk factors…”
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Deficiency of ATP13A2 leads to lysosomal dysfunction, α-synuclein accumulation, and neurotoxicity by Usenovic, Marija, Tresse, Emilie, Mazzulli, Joseph R, Taylor, J Paul, Krainc, Dimitri

“…The autophagy-lysosomal pathway plays an important role in the clearance of long-lived proteins and dysfunctional organelles. Lysosomal dysfunction has been…”
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Modeling neuronopathic storage diseases with patient-derived culture systems by Zunke, Friederike, Mazzulli, Joseph R.

“…Lysosomes are organelles involved in the degradation and recycling of macromolecules, and play a critical role in sensing metabolic information in the cell. A…”
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Increased Lysosomal Exocytosis Induced by Lysosomal Ca2+ Channel Agonists Protects Human Dopaminergic Neurons from α-Synuclein Toxicity by Tsunemi, Taiji, Perez-Rosello, Tamara, Ishiguro, Yuta, Yoroisaka, Asako, Jeon, Sohee, Hamada, Kana, Rammonhan, Malini, Wong, Yvette C, Xie, Zhong, Akamatsu, Wado, Mazzulli, Joseph R, Surmeier, D James, Hattori, Nobutaka, Krainc, Dimitri

“…The accumulation of misfolded proteins is a common pathological feature of many neurodegenerative disorders, including synucleinopathies such as Parkinson's…”
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Rescue of α-synuclein aggregation in Parkinson’s patient neurons by synergistic enhancement of ER proteostasis and protein trafficking by Stojkovska, Iva, Wani, Willayat Y., Zunke, Friederike, Belur, Nandkishore R., Pavlenko, Egor A., Mwenda, Nkatha, Sharma, Karan, Francelle, Laetitia, Mazzulli, Joseph R.

“…Neurodegenerative disorders are characterized by a collapse in proteostasis, as shown by the accumulation of insoluble protein aggregates in the brain…”
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Parkin and PINK1 Patient iPSC-Derived Midbrain Dopamine Neurons Exhibit Mitochondrial Dysfunction and α-Synuclein Accumulation by Chung, Sun Young, Kishinevsky, Sarah, Mazzulli, Joseph R., Graziotto, John, Mrejeru, Ana, Mosharov, Eugene V., Puspita, Lesly, Valiulahi, Parvin, Sulzer, David, Milner, Teresa A., Taldone, Tony, Krainc, Dimitri, Studer, Lorenz, Shim, Jae-won

“…Parkinson's disease (PD) is characterized by the selective loss of dopamine neurons in the substantia nigra; however, the mechanism of neurodegeneration in PD…”
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Activation of β-Glucocerebrosidase Reduces Pathological α-Synuclein and Restores Lysosomal Function in Parkinson's Patient Midbrain Neurons by Mazzulli, Joseph R, Zunke, Friederike, Tsunemi, Taiji, Toker, Nicholas J, Jeon, Sohee, Burbulla, Lena F, Patnaik, Samarjit, Sidransky, Ellen, Marugan, Juan J, Sue, Carolyn M, Krainc, Dimitri

“…Parkinson's disease (PD) is characterized by the accumulation of α-synuclein (α-syn) within Lewy body inclusions in the nervous system. There are currently no…”
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Impaired Autophagic-Lysosomal Fusion in Parkinson's Patient Midbrain Neurons Occurs through Loss of ykt6 and Is Rescued by Farnesyltransferase Inhibition by Pitcairn, Caleb, Murata, Naomi, Zalon, Annie J, Stojkovska, Iva, Mazzulli, Joseph R

“…Macroautophagy is a catabolic process that coordinates with lysosomes to degrade aggregation-prone proteins and damaged organelles. Loss of macroautophagy…”
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α-Synuclein: Multiple pathogenic roles in trafficking and proteostasis pathways in Parkinson’s disease by Zalon, Annie J., Quiriconi, Drew J., Pitcairn, Caleb, Mazzulli, Joseph R.

“…Parkinson’s disease (PD) is a common age-related neurodegenerative disorder characterized by the loss of dopaminergic neurons in the midbrain. A hallmark of…”
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Sirt1 mediates neuroprotection from mutant huntingtin by activation of the TORC1 and CREB transcriptional pathway by Jeong, Hyunkyung, Cohen, Dena E, Cui, Libin, Supinski, Andrea, Savas, Jeffrey N, Mazzulli, Joseph R, Yates, John R, Bordone, Laura, Guarente, Leonard, Krainc, Dimitri

“…Huntington's disease is a neurodegenerative disease caused by the accumulation of mutant HTT protein. Now, two groups led by Dimitri Krainc and Wenzhen Duan…”
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Dopamine induces soluble α-synuclein oligomers and nigrostriatal degeneration by Mor, Danielle E, Tsika, Elpida, Mazzulli, Joseph R, Gould, Neal S, Kim, Hanna, Daniels, Malcolm J, Doshi, Shachee, Gupta, Preetika, Grossman, Jennifer L, Tan, Victor X, Kalb, Robert G, Caldwell, Kim A, Caldwell, Guy A, Wolfe, John H, Ischiropoulos, Harry

“…Dopamine has long been thought to contribute to neurodegeneration in Parkinson's disease. The authors show that dopamine-induced neuron death in the substantia…”
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The hexosamine biosynthetic pathway rescues lysosomal dysfunction in Parkinson’s disease patient iPSC derived midbrain neurons by Wani, Willayat Y., Zunke, Friederike, Belur, Nandkishore R., Mazzulli, Joseph R.

“…Disrupted glucose metabolism and protein misfolding are key characteristics of age-related neurodegenerative disorders including Parkinson’s disease, however…”
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Nuclear aggregates of NONO/SFPQ and A-to-I-edited RNA in Parkinson's disease and dementia with Lewy bodies by Belur, Nandkishore R, Bustos, Bernabe I, Lubbe, Steven J, Mazzulli, Joseph R

“…Neurodegenerative diseases are commonly classified as proteinopathies that are defined by the aggregation of a specific protein. Parkinson's disease (PD) and…”
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