Zobrazeno 1 - 10
of 180
pro vyhledávání: '"Gary D. Housley"'
Autor:
Jeremy L. Pinyon, Georg vonJonquieres, Edward N. Crawford, Amr Al Abed, John M. Power, Matthias Klugmann, Cherylea J. Browne, David M. Housley, Andrew K. Wise, James B. Fallon, Robert K. Shepherd, John Y. Lin, Catherine McMahon, David McAlpine, Catherine S. Birman, Waikong Lai, Ya Lang Enke, Paul M. Carter, James F. Patrick, Robert D. Gay, Corinne Marie, Daniel Scherman, Nigel H. Lovell, Gary D. Housley
Publikováno v:
Advanced Science, Vol 11, Iss 30, Pp n/a-n/a (2024)
Abstract Viral vectors and lipofection‐based gene therapies have dispersion‐dependent transduction/transfection profiles that thwart precise targeting. The study describes the development of focused close‐field gene electrotransfer (GET) techno
Externí odkaz:
https://doaj.org/article/1f120ece41dd42df991a8e5de27489c2
Publikováno v:
Frontiers in Neuroscience, Vol 17 (2023)
Aminoacyl-tRNA synthetases (ARSs) play an essential role in protein synthesis, being responsible for ligating tRNA molecules to their corresponding amino acids in a reaction known as ‘tRNA aminoacylation’. Separate ARSs carry out the aminoacylati
Externí odkaz:
https://doaj.org/article/74ee165049d84cc4a75ed4f3764eaf56
Publikováno v:
Frontiers in Neuroscience, Vol 17 (2023)
Protein synthesis is a fundamental process that underpins almost every aspect of cellular functioning. Intriguingly, despite their common function, recessive mutations in aminoacyl-tRNA synthetases (ARSs), the family of enzymes that pair tRNA molecul
Externí odkaz:
https://doaj.org/article/80a5fd1bf33f4523b9bf2b149c7730ec
Autor:
Dominik Fröhlich, Elizabeth Kalotay, Georg von Jonquieres, Andre Bongers, Brendan Lee, Alexandra K. Suchowerska, Gary D. Housley, Matthias Klugmann
Publikováno v:
Frontiers in Molecular Neuroscience, Vol 15 (2022)
The leukodystrophy Canavan disease is a fatal white matter disorder caused by loss-of-function mutations of the aspartoacylase-encoding ASPA gene. There are no effective treatments available and experimental gene therapy trials have failed to provide
Externí odkaz:
https://doaj.org/article/537c8c0bb7154f5697810c52b8705019
Autor:
Jennie M. E. Cederholm, Kristina E. Parley, Chamini J. Perera, Georg von Jonquieres, Jeremy L. Pinyon, Jean-Pierre Julien, David K. Ryugo, Allen F. Ryan, Gary D. Housley
Publikováno v:
Frontiers in Neurology, Vol 13 (2022)
In the post-natal mouse cochlea, type II spiral ganglion neurons (SGNs) innervating the electromotile outer hair cells (OHCs) of the ‘cochlear amplifier' selectively express the type III intermediate filament peripherin gene (Prph). Immunolabeling
Externí odkaz:
https://doaj.org/article/6543568256984b84800d74441f2aa131
Publikováno v:
Frontiers in Cellular Neuroscience, Vol 15 (2021)
Central Nervous System (CNS) homeostasis and function rely on intercellular synchronization of metabolic pathways. Developmental and neurochemical imbalances arising from mutations are frequently associated with devastating and often intractable neur
Externí odkaz:
https://doaj.org/article/7d0746bbf6f1452bb45650d3b8f76802
Autor:
Dominik Fröhlich, Marisa I. Mendes, Andrew J. Kueh, Andre Bongers, Marco J. Herold, Gajja S. Salomons, Gary D. Housley, Matthias Klugmann
Publikováno v:
Frontiers in Cellular Neuroscience, Vol 14 (2021)
Hypomyelination with brain stem and spinal cord involvement and leg spasticity (HBSL) is a leukodystrophy caused by missense mutations of the aspartyl-tRNA synthetase-encoding gene DARS1. The clinical picture includes the regression of acquired motor
Externí odkaz:
https://doaj.org/article/2a471b43f5584d4da9624c90395f5e86
Publikováno v:
Frontiers in Cellular Neuroscience, Vol 14 (2021)
Aminoacyl-tRNA synthetases (ARSs) accurately charge tRNAs with their respective amino acids. As such, they are vital for the initiation of cytosolic and mitochondrial protein translation. These enzymes have become increasingly scrutinized in recent y
Externí odkaz:
https://doaj.org/article/64b1ddd3a3284c518b9b22ad3651923e
Autor:
Mian Bi, Amadeus Gladbach, Janet van Eersel, Arne Ittner, Magdalena Przybyla, Annika van Hummel, Sook Wern Chua, Julia van der Hoven, Wei S. Lee, Julius Müller, Jasneet Parmar, Georg von Jonquieres, Holly Stefen, Ernesto Guccione, Thomas Fath, Gary D. Housley, Matthias Klugmann, Yazi D. Ke, Lars M. Ittner
Publikováno v:
Nature Communications, Vol 8, Iss 1, Pp 1-15 (2017)
Excitotoxicity contributes to neuronal injury following stroke. Here the authors show that tau promotes excitotoxicity by a post-synaptic mechanism, involving site-specific control of ERK activation, in a mouse model of stroke.
Externí odkaz:
https://doaj.org/article/97fd15d6e4f1429dae0533cf6a3311e1
Autor:
Dominik Fröhlich, Alexandra K. Suchowerska, Ziggy H.T. Spencer, Georg von Jonquieres, Claudia B. Klugmann, Andre Bongers, Fabien Delerue, Holly Stefen, Lars M. Ittner, Thomas Fath, Gary D. Housley, Matthias Klugmann
Publikováno v:
Neurobiology of Disease, Vol 97, Iss , Pp 24-35 (2017)
Background: The recently diagnosed leukodystrophy Hypomyelination with Brain stem and Spinal cord involvement and Leg spasticity (HBSL) is caused by mutations of the cytoplasmic aspartyl-tRNA synthetase gene DARS. The physiological role of DARS in tr
Externí odkaz:
https://doaj.org/article/39cbdf69bfe24fa787ad07522d5df957