Optineurin Dysfunction in Amyotrophic Lateral Sclerosis: Why So Puzzling?
| Autor: | Marin Dominovic, Josip Peradinovic, Rozalija Šajn, Ivana Munitic, Andrea Markovinovic, Nikolina Prtenjaca |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
Necroptosis
Autophagy optineurin ALS General Medicine Golgi apparatus Biology medicine.disease General Biochemistry Genetics and Molecular Biology symbols.namesake symbols medicine Amyotrophic lateral sclerosis Signal transduction General Agricultural and Biological Sciences IRF3 Neuroscience Optineurin Interferon regulatory factors |
| Zdroj: | Periodicum biologorum Volume 121-122 Issue 1-2 |
| ISSN: | 1849-0964 0031-5362 |
| Popis: | Mutations in optineurin have been linked to amyotrophic lateral sclero- sis (ALS) a decade ago, but its exact role in the neurodegenerative process is still unclear. As a lysine 63 (K63)- and methionine (M1)-linked polyubiq- uitin-binding protein, optineurin has been reported to act as an adaptor in inflammatory signaling pathways mediated via nuclear factor kappa-light- chain-enhancer of activated B cells (NF-κB) and interferon regulatory fac- tor 3 (IRF3), as well as in membrane-associated trafficking events including autophagy, maintenance of the Golgi apparatus, and exocytosis. Other stud- ies have demonstrated its role in other processes such as regulation of mitosis, transcription, necroptosis and apoptosis. However, many of the reported effects in cell models have been proven difficult to reproduce in optineurin animal models, demonstrating the challenges of extrapolation between model systems. Knowing that multifunctional proteins present a “nightmare” for researchers, to help navigating through this field, we address the most common controversies, open questions, and artefacts related to optineurin and its role in pathogenesis of ALS and other neurodegenerative diseases. |
| Databáze: | OpenAIRE |
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