Zobrazeno 1 - 10
of 52
pro vyhledávání: '"Georg Von Jonquieres"'
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:
Laura A. Richards, Ashu Kumari, Kathy Knezevic, Julie AI Thoms, Georg von Jonquieres, Christine E. Napier, Zara Ali, Rosemary O’Brien, Jonathon Marks-Bluth, Michelle F. Maritz, Hilda A Pickett, Jonathan Morris, John E. Pimanda, Karen L. MacKenzie
Publikováno v:
Haematologica, Vol 105, Iss 6 (2020)
Telomerase is a ribonucleoprotein complex that maintains the length and integrity of telomeres, and thereby enables cellular proliferation. Understanding the regulation of telomerase in hematopoietic cells is relevant to the pathogenesis of leukemia,
Externí odkaz:
https://doaj.org/article/ee07ad5c6c0249799f5927f0f6b11e13
Autor:
Chelsea N. Brown, Elissa K. Fultz, Sami Ferdousian, Sarina Rogers, Elijah Lustig, Ariana Page, John R. Shahin, Daniel M. Flaherty, Georg Von Jonquieres, Camron D. Bryant, Tod E. Kippin, Karen K. Szumlinski
Publikováno v:
Frontiers in Psychiatry, Vol 11 (2020)
Problems associated with the abuse of amphetamine-type stimulants, including methamphetamine (MA), pose serious health and socioeconomic issues world-wide. While it is well-established that MA’s psychopharmacological effects involve interactions wi
Externí odkaz:
https://doaj.org/article/bc6c7e4790e748cc85b9c6cab48038e9
Autor:
Kim L. Powell, Xavier Fitzgerald, Claire Shallue, Valentina Jovanovska, Matthias Klugmann, Georg Von Jonquieres, Terence J. O'Brien, Margaret J. Morris
Publikováno v:
Neurobiology of Disease, Vol 113, Iss , Pp 23-32 (2018)
Neuropeptide Y (NPY) is an important 36 amino acid peptide that is abundantly expressed in the mammalian CNS and is known to be an endogenous modulator of seizure activity, including in rat models of Genetic Generalised Epilepsy (GGE) with absence se
Externí odkaz:
https://doaj.org/article/6b6f58fc2d804e83905ae1080be7ef5e
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
Autor:
David M. Housley, Jeremy L. Pinyon, Georg von Jonquieres, Chamini J. Perera, Michael Smout, Michael J. Liddell, Ernest A. Jennings, David Wilson, Gary D. Housley
Publikováno v:
Biomolecules, Vol 10, Iss 4, p 617 (2020)
Scorpion venoms are a rich source of bioactive molecules, but characterisation of toxin peptides affecting cytosolic Ca2+, central to cell signalling and cell death, is limited. We undertook a functional screening of the venom of the Australian scorp
Externí odkaz:
https://doaj.org/article/a13ed9de228c4168badb65fd4b07b9a5
Autor:
Dominik Fröhlich, Alexandra K. Suchowerska, Carola Voss, Ruojie He, Ernst Wolvetang, Georg von Jonquieres, Cas Simons, Thomas Fath, Gary D. Housley, Matthias Klugmann
Publikováno v:
Frontiers in Molecular Neuroscience, Vol 11 (2018)
Translation of mRNA into protein is an evolutionarily conserved, fundamental process of life. A prerequisite for translation is the accurate charging of tRNAs with their cognate amino acids, a reaction catalyzed by specific aminoacyl-tRNA synthetases
Externí odkaz:
https://doaj.org/article/86ef7b7ec44c421cb4122f75498e7251