Results 151 to 160 of about 67,038 (301)

The Race to Salvage Glucocerebrosidase: Understanding Small‐Molecule Therapies for GBA1‐Associated Parkinsonism

Movement Disorders, EarlyView.
Variants in GBA1, the gene encoding the lysosomal enzyme glucocerebrosidase, cause Gaucher disease and confer an increased risk for parkinsonism. Strategies using small molecules can improve the function of glucocerebrosidase in lysosomes. A clear understanding of the mechanism‐of‐action of these compounds will facilitate development of GBA1‐modulating
Mark J. Henderson, Tiffany C. Chen, Logan M. Glasstetter, Yu Chen, Juan J. Marugan, Ellen Sidransky   +5 more
wiley   +1 more source

Discordance of Dopaminergic Dysfunction and Subcortical Atrophy by α‐Synuclein Status in Sporadic and Genetic Parkinson's Disease

Movement Disorders, EarlyView.
Abstract Background Parkinson's disease (PD) is characterized by predominantly neuronal α‐synuclein pathology and dopaminergic dysfunction. Cerebrospinal fluid (CSF) seeding amplification assays (SAA) detect α‐synuclein aggregates in vivo, but not all patients with PD have a positive SAA.
Michael Tran Duong, Sandhitsu R. Das, Pulkit Khandelwal, Joaquin A. Vizcarra, Yue Li, Long Xie, Paul A. Yushkevich, Leslie M. Shaw, Jacob G. Dubroff, Parkinson's Progression Markers Initiative, Kenneth Marek, Shirley Lasch, Caroline Tanner, Tanya Simuni, Christopher Coffey, Karl Kieburtz, Renee Wilson, Brit Mollenhauer, Site Investigator, Douglas Galasko, Site Investigator, Tatiana Foroud, Lana Chahine, Andrew Siderowf, John Seibyl, Arthur Toga, Andrew Singleton, Daniel Weintraub, John Trojanowski, Leslie Shaw, Duygu Tosun‐Turgut, Kathleen Poston, Susan Bressman, Kalpana M. Merchant, Werner Poewe, Todd Sherer, Sohini Chowdhury, Mark Frasier, Catherine Kopil, Anna Naito, Vanessa Arnedo, Ray Dorsey, Cynthia Casaceli, Imaging Core, Nichole Daegele, Justin Albani1 Statistics Core, Chelsea Caspell‐Garcia, Liz Uribe, Eric Foster, Jeff Long, Nick Seedorff, Karen Crawford, Danielle Elise Smith, Paola Casalin, Giulia Malferrari, Cheryl Halter, David Russell, Stewart Factor, Penelope Hogarth, David Standaert, Amy Amara, Robert Hauser, Joseph Jankovic, Matthew Stern, Shu‐Ching Hu, Gretchen Todd, Rachel Saunders‐Pullman, Irene Richard, Marie H Saint‐Hilaire, Klaus Seppi, Holly Shill, Hubert Fernandez, Claudia Trenkwalder, Wolfgang Oertel, Daniela Berg, Kathrin Brockman, Isabel Wurster, Liana Rosenthal, Yen Tai, Nicola Pavese, Paolo Barone, Stuart Isaacson, Alberto Espay, Dominic Rowe, Melanie Brandabur, James Tetrud, Grace Liang, Alex Iranzo, Eduardo Tolosa, Karen Marder, Maria de Arriba Sanchez, Leonidis Stefanis, Maria Jose Marti, Javier Ruiz Martinez, Jean‐Christophe Corvol, Jan O Assly, Salima Brillman, Nir Giladi, Debra Smejdir, Julia Pelaggi, Farah Kausar, Linda Rees, Barbara Sommerfield, Madeline Cresswell, Courtney Blair, Karen Williams, Grace Zimmerman, Stephanie Guthrie, Ashlee Rawlins, Leigh Donharl, Christine Hunter, Baochan Tran, Abigail Darin, Carly Linder, Marne Baca, Heli Venkov, Cathi‐Ann Thomas, Raymond James, Beatrice Heim, Paul Deritis, Fabienne Sprenger, Deborah Raymond, Diana Willeke, Zoran Obradov, Jennifer Mule, Nancy Monahan, Katharina Gauss, Deborah Fontaine, Daniel Szpak, Arita McCoy, Becky Dunlop, Laura Marie Payne, Susan Ainscough, Lisbeth Carvajal, Rebecca Silverstein, Kristy Espay, Madelaine Ranola, Elisabet Mondragon Rezola, Helen Mejia Santana, Maria Stamelou, Alicia Garrido, Stephanie Carvalho, Anne Grete Kristiansen, Krista Specketer, Anat Mirlman, Maurizio Facheris, Holly Soares, Mark A. Mintun, Jesse Cedarbaum, Peggy Taylor, Danna Jennings, Lawrence Slieker, Brian McBride, Colin Watson, Etienne Montagut, Zulfiqar Haider Sheikh, Baris Bingol, Remi Forrat, Pablo Sardi, Tanya Fischer, Alastair D. Reith, Jan Egebjerg, Lone Frydelund Larsen, Nathalie Breysse, Didier Meulien, Barbara Saba, Vera Kiyasova, Chris Min, Thomas McAvoy, Robert Umek, Philip Iredale, Jeremy Edgerton, Susan De Santi, Christian Czech, Frank Boess, Jeffrey Sevigny, Thomas Kremer, Igor Grachev, Kaplana Merchant, Andreja Avbersek, Pierandrea Muglia, Alexandra Stewart, Rene Prashad, Johannes Taucher, Andrew Siderowf, David A. Wolk, Ilya M. Nasrallah   +186 more
wiley   +1 more source

Inhibitory effect of Scutellaria pinnatifida Extracts on the Alpha synuclein Cytotoxicity

Journal of Medicinal Plants, 2019
Many of neurodegenerative diseases (NDs) are associated with formation of the protein aggregates called amyloidal fibrils. Fibrillization of alpha-synuclein seems to be a key stage in the creation and progression of NDs and finding the compounds that ...
Mahdieh Sashourpour, Dina Morshedi, Saber Zahri, Farhang Ali akbari, Marzieh Fottovat, Tayebeh Rajabian   +5 more
doaj  

Perampanel Blocks Transsynaptic α‐Synuclein Propagation and Neurodegeneration in a Mouse Model of Lewy Body Disease

Movement Disorders, EarlyView.
Abstract Background Transsynaptic α‐synuclein propagation plays a crucial role in the progression of Lewy body disease. We previously demonstrated that an α‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid receptor antagonist, perampanel, blocks neuronal uptake of α‐synuclein preformed fibrils (PFFs) in an activity‐dependent manner.
Jun Ueda, Norihito Uemura, Jiarui Chang, Tetsuya Hirato, Tomoyuki Ishimoto, Miki Oono, Shuichi Matsuzawa, Riki Matsumoto, Hodaka Yamakado, Ryosuke Takahashi   +9 more
wiley   +1 more source

A broken alpha -helix in folded alpha -Synuclein.

The Journal of biological chemistry, 2003
alpha-Synuclein is a small cytosolic protein of presynaptic nerve terminals composed of seven 11-residue repeats and a hydrophilic tail. alpha-Synuclein misfolding and dysfunction may contribute to the pathogenesis of Parkinson's disease and neurodegenerative dementias, but its normal folding and function are unknown.
Chandra, S., Chen, X., Rizo, J., Jahn, R., Suedhof, T.   +4 more
openaire   +2 more sources

Types of Pain in Multiple System Atrophy

Movement Disorders, EarlyView.
Abstract Background Pain affects up to 87% of people with multiple system atrophy (MSA), but it remains unclear which types of pain contribute most to the overall burden. Objective To estimate the frequency of different types of pain in MSA individuals.
Nicole Campese, Mubasher A. Qamar, Maria Alexandra Chiriac, Georg Göbel, Julia Wanschitz, Andreas Schlager, Bianca Caliò, Fabian Leys, Pam Bower, Laura Zamarian, Anette Schrag, Roy Freeman, Horacio Kaufmann, Roberta Granata, Stefan Kiechl, Werner Poewe, Klaus Seppi, Gregor Wenning, K. Ray Chaudhuri, Alessandra Fanciulli   +19 more
wiley   +1 more source

Clinical and Imaging Characteristics of Parkinson's Disease with Negative Alpha‐Synuclein Seed Amplification Assay

Movement Disorders, EarlyView.
Abstract Background The cerebrospinal fluid alpha‐synuclein seed amplification assay (CSFasynSAA) detects alpha‐synuclein aggregation in over 90% of individuals with sporadic PD (sPD). However, the clinical characteristics of sPD with negative CSFasynSAA remain undefined.
Sarah M. Brooker, Jacopo Pasquini, Seung Ho Choi, David‐Erick Lafontant, Seyed‐Mohammad Fereshtehnejad, Yashar Zeighami, Piergiorgio Grillo, Giulietta M. Riboldi, Houman Azizi, Roqaie Moqadam, Un Jung Kang, Kelly N.H. Nudelman, Andrew Siderowf, Caroline M. Tanner, Thomas F. Tropea, Tatiana Foroud, Lana M. Chahine, Brit Mollenhauer, Kalpana M. Merchant, Douglas Galasko, Christopher S. Coffey, Roseanne D. Dobkin, Ethan G. Brown, Roy N. Alcalay, Daniel Weintraub, Kenneth Marek, Tanya Simuni, Paulina Gonzalez‐Latapi, Nicola Pavese, Kathleen L. Poston, the Parkinson's Progression Markers Initiative   +30 more
wiley   +1 more source

Early Autonomic Burden in Prodromal Parkinson's Disease Predicts Cognitive Impairment

Movement Disorders, EarlyView.
Abstract Background Autonomic dysfunction is a known contributor to cognitive impairment in Parkinson's disease (PD), but its impact during prodromal stage is unknown. Objective The aim was to determine whether early autonomic burden predicts incident cognitive impairment in prodromal PD.
A. Enrique Martinez‐Nunez, Kelly A. Mills, Joseph Seemiller, Christopher B. Morrow, Gregory M. Pontone, Abhimanyu Mahajan   +5 more
wiley   +1 more source

Plasma Glucosylsphingosine in GBA1 E365K, N409S, and L483P Heterozygous Mutation Carriers

Movement Disorders, EarlyView.
Abstract Background GBA1 encodes the lysosomal enzyme glucocerebrosidase, with key substrates that include glucosylceramide and glucosylsphingosine. The E365K variant is the most common variant in GBA1 that is associated with Parkinson's disease (PD) but is not associated with Gaucher disease.
Julian Agin‐Liebes, Alexander Haimovich, Rachel Griep, Nathan Galen Hatcher, Lihang Yao, Cheryl Waters, Shalini Padmanabhan, Roy N. Alcalay   +7 more
wiley   +1 more source

Quantitative Analysis of Locus Coeruleus Neurons and Thalamic Noradrenergic Axons in a Progressive 1‐Methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine Monkey Model of Parkinson's Disease

Movement Disorders, EarlyView.
Abstract Background Parkinson's disease is mainly characterized by dopaminergic neurodegeneration in the substantia nigra pars compacta (SNc) and α‐synuclein accumulation. The locus coeruleus (LC) is also affected in Parkinson's disease and linked to some nonmotor symptoms, but the extent and timing of its degeneration remain unclear.
Megan Carrillo, Nagore Hernández‐Pinedo, Josefa Zaldivar‐Diez, Isabel Pérez‐Santos, José A. Obeso, Carmen Cavada, Javier Blesa   +6 more
wiley   +1 more source