Nuclear Factor Erythroid-Related Factor 2 Signaling in the Neuropathophysiology of Inherited Metabolic Disorders
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TLDR
In this paper, a review of the current knowledge about Nrf2 signaling dysregulation observed in X-linked adrenoleukodystrophy, glutaric acidemia type I, hyperhomocysteinemia and Friedreich's ataxia is presented.Citations
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A Pivotal Role of Nrf2 in Neurodegenerative Disorders: A New Way for Therapeutic Strategies
TL;DR: This review describes the influence of Nrf2 on OS and in what way NRF2 regulates antioxidant defense for neurodegenerative conditions and evaluates recent research and evidence for a beneficial and potential role of specific Nrf 2 activator compounds as therapeutic agents.
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Nrf2 Pathway in Huntington’s Disease (HD): What Is Its Role?
TL;DR: In this paper , the authors present the scientific literature supporting the role of Nrf2 in Huntington's disease and the potential prophylactic and therapeutic role of this compound, which triggers an endogenous antioxidant pathway activated in different neurodegenerative disorders.
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The Role of the NRF2 Pathway in Maintaining and Improving Cognitive Function
TL;DR: This review focused on the evidence supporting a role for NRF2 activation in maintaining and improving cognitive function and how its activation can be used to elicit neuroprotection and lead to cognitive enhancement.
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Disturbance of Mitochondrial Dynamics, Endoplasmic Reticulum–Mitochondria Crosstalk, Redox Homeostasis, and Inflammatory Response in the Brain of Glutaryl-CoA Dehydrogenase-Deficient Mice: Neuroprotective Effects of Bezafibrate
TL;DR: In vivo treatment with the pan-PPAR agonist bezafibrate normalized alterations in redox homeostasis, inflammatory response, mitochondrial biogenesis and dynamics, endoplasmic reticulum (ER)-mitochondria crosstalk, and ER stress in the brain of GCDH-deficient and wild-type mice fed a high Lys chow, suggesting novel pathomechanisms may be involved in GA1 neuropathology.
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Glucoraphanin Triggers Rapid Antidepressant Responses in a Rat Model of Beta Amyloid-Induced Depressive-like Behaviour
Paolo Tucci,Maria Bove,Vladyslav Volodymyrovych Sikora,Stefania Dimonte,Maria Grazia Morgese,Stefania Schiavone,Lorenzo Di Cesare Mannelli,Carla Ghelardini,Luigia Trabace +8 more
TL;DR: Investigation of the effect of GRA in naïve and in sAβ-1-42-treated rats by using the FST found that GRA rapidly reverting depressive-like behaviour, together with biochemical and neurochemical alterations, might represent a safe and natural candidate for the treatment of depression.
References
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Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion
Victoria Campuzano,Laura Montermini,María Dolores Moltó,Luigi Pianese,Mireille Cossée,F Cavalcanti,Eugenia Monros,François Rodius,Franck Duclos,Antonella Monticelli,Federico Zara,Joaquín Cañizares,Hana Koutnikova,Sanjay I. Bidichandani,Cinzia Gellera,Alexis Brice,Paul Trouillas,Giuseppe De Michele,Alessandro Filla,Rosa de Frutos,Francisco Palau,Pragna Patel,Stefano Di Donato,Jean-Louis Mandel,Sergio Cocozza,Michel Koenig,Massimo Pandolfo +26 more
TL;DR: A few FRDA patients were found to have point mutations in X25, but the majority were homozygous for an unstable GAA trinucleotide expansion in the first X25 intron.
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Oxidative stress and neurodegeneration: where are we now?
TL;DR: After a long lag period, therapeutic and other interventions based on a knowledge of redox biology are on the horizon for at least some of the neurodegenerative diseases.
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The selective autophagy substrate p62 activates the stress responsive transcription factor Nrf2 through inactivation of Keap1
Masaaki Komatsu,Hirofumi Kurokawa,Satoshi Waguri,Keiko Taguchi,Akira Kobayashi,Yoshinobu Ichimura,Yoshinobu Ichimura,Yu-shin Sou,Yu-shin Sou,Izumi Ueno,Ayako Sakamoto,Kit I. Tong,Mihee Kim,Yasumasa Nishito,Shun-ichiro Iemura,Tohru Natsume,Takashi Ueno,Eiki Kominami,Hozumi Motohashi,Keiji Tanaka,Masayuki Yamamoto +20 more
TL;DR: The findings indicate that the pathological process associated with p62 accumulation results in hyperactivation of Nrf2 and delineates unexpected roles of selective autophagy in controlling the transcription of cellular defence enzyme genes.
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Nrf2 signaling pathway: Pivotal roles in inflammation.
TL;DR: The members of the Keap1/Nrf2/ARE signal pathway and its downstream genes, the effects of this pathway on animal models of inflammatory diseases, and crosstalk with the NF-κB pathway are discussed.
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Clinical and Genetic Abnormalities in Patients with Friedreich's Ataxia
Alexandra Durr,Mireille Cossée,Yves Agid,Victoria Campuzano,Claude Mignard,C. Penet,Jean-Louis Mandel,Alexis Brice,Michel Koenig +8 more
TL;DR: The clinical spectrum of Friedreich's ataxia is broader than previously recognized, and the direct molecular test for the GAA expansion on chromosome 9 is useful for diagnosis, determination of prognosis, and genetic counseling.