Search Results - "Gorecki, Dariusz C"

Biomimetic generation of the strongest known biomaterial found in limpet tooth by Rumney, Robin M. H., Robson, Samuel C., Kao, Alexander P., Barbu, Eugen, Bozycki, Lukasz, Smith, James R., Cragg, Simon M., Couceiro, Fay, Parwani, Rachna, Tozzi, Gianluca, Stuer, Michael, Barber, Asa H., Ford, Alex T., Górecki, Dariusz C.

“…The biomaterial with the highest known tensile strength is a unique composite of chitin and goethite (α-FeO(OH)) present in teeth from the Common Limpet (…”
Get full text

Myogenesis modelled by human pluripotent stem cells: a multi‐omic study of Duchenne myopathy early onset by Mournetas, Virginie, Massouridès, Emmanuelle, Dupont, Jean‐Baptiste, Kornobis, Etienne, Polvèche, Hélène, Jarrige, Margot, Dorval, Alan R.L., Gosselin, Maxime R.F., Manousopoulou, Antigoni, Garbis, Spiros D., Górecki, Dariusz C., Pinset, Christian

“…Background Duchenne muscular dystrophy (DMD) causes severe disability of children and death of young men, with an incidence of approximately 1/5000 male…”
Get full text

A novel mechanism of autophagic cell death in dystrophic muscle regulated by P2RX7 receptor large-pore formation and HSP90 by Young, Christopher NJ, Sinadinos, Anthony, Lefebvre, Alexis, Chan, Philippe, Arkle, Stephen, Vaudry, David, Gorecki, Dariusz C

“…P2RX7 is an ATP-gated ion channel, which can also exhibit an open state with a considerably wider permeation. However, the functional significance of the…”
Get full text

Loss of full-length dystrophin expression results in major cell-autonomous abnormalities in proliferating myoblasts by Gosselin, Maxime R F, Mournetas, Virginie, Borczyk, Malgorzata, Verma, Suraj, Occhipinti, Annalisa, Róg, Justyna, Bozycki, Lukasz, Korostynski, Michal, Robson, Samuel C, Angione, Claudio, Pinset, Christian, Gorecki, Dariusz C

“…Duchenne muscular dystrophy (DMD) affects myofibers and muscle stem cells, causing progressive muscle degeneration and repair defects. It was unknown whether…”
Get full text

Expression, assembly and function of novel C‐terminal truncated variants of the mouse P2X7 receptor: re‐evaluation of P2X7 knockouts by Masin, Marianela, Young, Christopher, Lim, KoiNi, Barnes, Sara J, Xu, Xing Jian, Marschall, Viola, Brutkowski, Wojciech, Mooney, Elizabeth R, Gorecki, Dariusz C, Murrell‐Lagnado, Ruth

“…BACKGROUND AND PURPOSE Splice variants of P2X7 receptor transcripts contribute to the diversity of receptor‐mediated responses. Here, we investigated…”
Get full text

Important role of matrix metalloproteinase 9 in epileptogenesis by Wilczynski, Grzegorz M, Konopacki, Filip A, Wilczek, Ewa, Lasiecka, Zofia, Gorlewicz, Adam, Michaluk, Piotr, Wawrzyniak, Marcin, Malinowska, Monika, Okulski, Pawel, Kolodziej, Lukasz R, Konopka, Witold, Duniec, Kamila, Mioduszewska, Barbara, Nikolaev, Evgeni, Walczak, Agnieszka, Owczarek, Dorota, Gorecki, Dariusz C, Zuschratter, Werner, Ottersen, Ole Petter, Kaczmarek, Leszek

“…Temporal lobe epilepsy (TLE) is a devastating disease in which aberrant synaptic plasticity plays a major role. We identify matrix metalloproteinase (MMP) 9 as…”
Get full text

Zidovudine ameliorates pathology in the mouse model of Duchenne muscular dystrophy via P2RX7 purinoceptor antagonism by Al-Khalidi, Rasha, Panicucci, Chiara, Cox, Paul, Chira, Natalia, Róg, Justyna, Young, Christopher N J, McGeehan, Rhiannon E, Ambati, Kameshwari, Ambati, Jayakrishna, Zabłocki, Krzysztof, Gazzerro, Elisabetta, Arkle, Stephen, Bruno, Claudio, Górecki, Dariusz C

“…Duchenne muscular dystrophy (DMD) is the most common inherited muscle disorder that causes severe disability and death of young men. This disease is…”
Get full text

Mutation in dystrophin-encoding gene affects energy metabolism in mouse myoblasts by Onopiuk, Marta, Brutkowski, Wojciech, Wierzbicka, Katarzyna, Wojciechowska, Sylwia, Szczepanowska, Joanna, Fronk, Jan, Lochmüller, Hanns, Górecki, Dariusz C., Zabłocki, Krzysztof

“…Duchenne Muscular Dystrophy is characterized by severe defects in differentiated muscle fibers, including abnormal calcium homeostasis and impaired cellular…”
Get full text

The α-dystrobrevins play a key role in maintaining the structure and function of the extracellular matrix-significance for protein elimination failure arteriopathies by Sharp, Matthew MacGregor, Cassidy, Jordan, Thornton, Thomas, Lyles, James, Keable, Abby, Gatherer, Maureen, Yasui, Masato, Abe, Yoichiro, Shibata, Shinsuke, Weller, Roy O, Górecki, Dariusz C, Carare, Roxana O

“…The extracellular matrix (ECM) of the cerebral vasculature provides a pathway for the flow of interstitial fluid (ISF) and solutes out of the brain by…”
Get full text

Knockdown of MLC1 in primary astrocytes causes cell vacuolation: A MLC disease cell model by Duarri, Anna, Lopez de Heredia, Miguel, Capdevila-Nortes, Xavier, Ridder, Margreet C, Montolio, Marisol, López-Hernández, Tania, Boor, Ilja, Lien, Chun-Fu, Hagemann, Tracy, Messing, Albee, Gorecki, Dariusz C, Scheper, Gert C, Martínez, Albert, Nunes, Virginia, van der Knaap, Marjo S, Estévez, Raúl

“…Abstract Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare type of leukodystrophy, in the majority of cases caused by mutations in the…”
Get full text

P2X7 purinoceptor as a therapeutic target in muscular dystrophies by Górecki, Dariusz C

“…•Inflammation is a pathological hallmark of muscular dystrophies.•Dystrophin and/or sarcoglycans loss alter eATP degradation and P2X7 signalling in…”
Get full text

Disrupted Calcium Homeostasis in Duchenne Muscular Dystrophy: A Common Mechanism behind Diverse Consequences by Zabłocka, Barbara, Górecki, Dariusz C, Zabłocki, Krzysztof

“…Duchenne muscular dystrophy (DMD) leads to disability and death in young men. This disease is caused by mutations in the gene encoding diverse isoforms of…”
Get full text

The Role of P2X7 Purinoceptors in the Pathogenesis and Treatment of Muscular Dystrophies by Zabłocki, Krzysztof, Górecki, Dariusz C

“…Muscular dystrophies are inherited neuromuscular diseases, resulting in progressive disability and often affecting life expectancy. The most severe, common…”
Get full text

Investigating the Involvement of C−X−C Motif Chemokine 5 and P2X7 Purinoceptor in Ectopic Calcification in Mouse Models of Duchenne Muscular Dystrophy by Rumney, Robin M. H., Pomeroy, Joanna, Górecki, Dariusz C.

“…ABSTRACT Ectopic calcification of myofibers is an early pathogenic feature in patients and animal models of Duchenne muscular dystrophy (DMD). In previous…”
Get full text

Primary mouse myoblast metabotropic purinoceptor profiles and calcium signalling differ with their muscle origin and are altered in mdx dystrophinopathy by Róg, Justyna, Oksiejuk, Aleksandra, Górecki, Dariusz C., Zabłocki, Krzysztof

“…Mortality of Duchenne Muscular Dystrophy (DMD) is a consequence of progressive wasting of skeletal and cardiac muscle, where dystrophinopathy affects not only…”
Get full text

Automated multi‐sample DNA extraction for genotyping live Xenopus embryos by Alles, Narmadha, Guille, Matthew, Górecki, Dariusz C.

“…Background Xenopus frogs are used extensively for modeling genetic diseases owing to characteristics such as the abundance of eggs combined with their large…”
Get full text

Engineering butylglyceryl-modified polysaccharides towards nanomedicines for brain drug delivery by Bostanudin, Mohammad F., Lalatsa, Aikaterini, Górecki, Dariusz C., Barbu, Eugen

“…•Amphiphilic guar gum, pullulan, and chitosan easily prepared with n-butylglycidyl ether.•Formulation into nanomedicines requires specific method…”
Get full text

P2RX7 Purinoceptor as a Therapeutic Target-The Second Coming? by Young, Chris N J, Górecki, Dariusz C

“…The P2RX7 receptor is a unique member of a family of extracellular ATP (eATP)-gated ion channels expressed in immune cells, where its activation triggers the…”
Get full text

β -dystrobrevin, a Member of the Dystrophin-Related Protein Family by Blake, Derek J., Nawrotzki, Ralph, Loh, Nellie Y., Gorecki, Dariusz C., Davies, Kay E.

“…The importance of dystrophin and its associated proteins in normal muscle function is now well established. Many of these proteins are expressed in nonmuscle…”
Get full text

P2RX7 purinoceptor: a therapeutic target for ameliorating the symptoms of duchenne muscular dystrophy by Sinadinos, Anthony, Young, Christopher N J, Al-Khalidi, Rasha, Teti, Anna, Kalinski, Paweł, Mohamad, Shafini, Floriot, Léonore, Henry, Tiphaine, Tozzi, Gianluca, Jiang, Taiwen, Wurtz, Olivier, Lefebvre, Alexis, Shugay, Mikhail, Tong, Jie, Vaudry, David, Arkle, Stephen, doRego, Jean-Claude, Górecki, Dariusz C

“…Duchenne muscular dystrophy (DMD) is the most common inherited muscle disease, leading to severe disability and death in young men. Death is caused by the…”
Get full text