Εξειδίκευση τύπου :	Άρθρο σε επιστημονικό περιοδικό
Τίτλος:	Protein aggregation and therapeutic strategies in SOD1- and TDP-43- linked ALS
Δημιουργός/Συγγραφέας:	Tsekrekou, Maria Giannakou, Maria Papanikolopoulou, Katerina [EL] Σκρέτας, Γιώργος[EN] Skretas, George
Ημερομηνία:	2024
Γλώσσα:	Αγγλικά
ISSN:	2296-889X
DOI:	10.3389/fmolb.2024.1383453
Άλλο:	38855322
Περίληψη:	Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with severe socio-economic impact. A hallmark of ALS pathology is the presence of aberrant cytoplasmic inclusions composed of misfolded and aggregated proteins, including both wild-type and mutant forms. This review highlights the critical role of misfolded protein species in ALS pathogenesis, particularly focusing on Cu/Zn superoxide dismutase (SOD1) and TAR DNA-binding protein 43 (TDP-43), and emphasizes the urgent need for innovative therapeutic strategies targeting these misfolded proteins directly. Despite significant advancements in understanding ALS mechanisms, the disease remains incurable, with current treatments offering limited clinical benefits. Through a comprehensive analysis, the review focuses on the direct modulation of the misfolded proteins and presents recent discoveries in small molecules and peptides that inhibit SOD1 and TDP-43 aggregation, underscoring their potential as effective treatments to modify disease progression and improve clinical outcomes.
Τίτλος πηγής δημοσίευσης:	Frontiers in molecular biosciences
Τόμος/Κεφάλαιο:	11
Θεματική Κατηγορία:	[EL] Βιοτεχνολογία[EN] Biotechnology [EL] Νευροεπιστήμη. Βιολογική ψυχιατρική. Νευροψυχιατρική[EN] Neurosciences. Biological psychiatry. Neuropsychiatry [EL] Βιοχημεία[EN] Biochemistry [EL] Θεραπευτική. Φαρμακολογία[EN] Therapeutics.Pharmacology
Λέξεις-Κλειδιά:	SOD1 TDP-43 amyotrophic lateral sclerosis neurotoxicity protein aggregation protein misfolding therapeutics
EU Grant:	ProMiDis Strengthening Human Resources Research Potential via Doctorate Research–2nd Cycle Twin4Promis Boost4Bio
EU Grant identifier:	819934 MIS 5000432 No. 101079363 No. 101087471
Κάτοχος πνευματικών δικαιωμάτων:	© 2024 Tsekrekou, Giannakou, Papanikolopoulou and Skretas
Όροι και προϋποθέσεις δικαιωμάτων:	This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
Ηλεκτρονική διεύθυνση στον εκδότη (link):	https://doi.org/10.3389/fmolb.2024.1383453
Εμφανίζεται στις συλλογές:	Ινστιτούτο Χημικής Βιολογίας - Επιστημονικό έργο