Antioxidant and Anti-Inflammatory Defenses in Huntington’s Disease: Roles of NRF2 and PGC-1α, and Therapeutic Strategies DOI Creative Commons
Francesco D’Egidio,

Elvira Qosja,

Fabrizio Ammannito

и другие.

Life, Год журнала: 2025, Номер 15(4), С. 577 - 577

Опубликована: Апрель 1, 2025

Huntington’s disease (HD) is a detrimental neurodegenerative caused by the expansion of CAG triplet in HTT gene. This mutation leads to production mutant Huntingtin (Htt) protein with toxic gain-of-function. The mHtt responsible several ways for establishment an intricate pathogenetic scenario affected cells, particularly HD neurons. Among features HD, oxidative stress plays relevant role progression at cellular level. Mitochondrial dysfunction, bioenergetic deficits, Reactive Oxygen Species (ROS) production, neuroinflammation, and general reduction antioxidant levels are all involved promotion environment, eventually causing cell death. Nonetheless, neuronal cells exert molecules build up defense mechanisms. Key components these defensive mechanisms nuclear factor erythroid 2-related 2 (NRF2) peroxisome proliferator-activated receptor gamma coactivator-1 α (PGC-1α). Thus, this review aims describe involvement exploring roles NRF2 PGC-1α, crucial actors play. Finally, therapeutic strategies targeting such markers discussed.

Язык: Английский

Antioxidant and Anti-Inflammatory Defenses in Huntington’s Disease: Roles of NRF2 and PGC-1α, and Therapeutic Strategies DOI Creative Commons
Francesco D’Egidio,

Elvira Qosja,

Fabrizio Ammannito

и другие.

Life, Год журнала: 2025, Номер 15(4), С. 577 - 577

Опубликована: Апрель 1, 2025

Huntington’s disease (HD) is a detrimental neurodegenerative caused by the expansion of CAG triplet in HTT gene. This mutation leads to production mutant Huntingtin (Htt) protein with toxic gain-of-function. The mHtt responsible several ways for establishment an intricate pathogenetic scenario affected cells, particularly HD neurons. Among features HD, oxidative stress plays relevant role progression at cellular level. Mitochondrial dysfunction, bioenergetic deficits, Reactive Oxygen Species (ROS) production, neuroinflammation, and general reduction antioxidant levels are all involved promotion environment, eventually causing cell death. Nonetheless, neuronal cells exert molecules build up defense mechanisms. Key components these defensive mechanisms nuclear factor erythroid 2-related 2 (NRF2) peroxisome proliferator-activated receptor gamma coactivator-1 α (PGC-1α). Thus, this review aims describe involvement exploring roles NRF2 PGC-1α, crucial actors play. Finally, therapeutic strategies targeting such markers discussed.

Язык: Английский

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