Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities

SJ Tabrizi, MD Flower, CA Ross, EJ Wild - Nature Reviews Neurology, 2020 - nature.com
Huntington disease (HD) is a neurodegenerative disease caused by CAG repeat expansion
in the huntingtin gene (HTT) and involves a complex web of pathogenic mechanisms …

PINK1/Parkin-mediated mitophagy in neurodegenerative diseases

J Li, D Yang, Z Li, M Zhao, D Wang, Z Sun, P Wen… - Ageing research …, 2023 - Elsevier
Mitochondria play key roles in bioenergetics, metabolism, and signaling; therefore, stable
mitochondrial function is essential for cell survival, particularly in energy-intensive neuronal …

Caspase-9: a multimodal therapeutic target with diverse cellular expression in human disease

MI Avrutsky, CM Troy - Frontiers in pharmacology, 2021 - frontiersin.org
Caspase-9, a cysteine-aspartic protease known for its role as an initiator of intrinsic
apoptosis, regulates physiological cell death and pathological tissue degeneration. Its …

ALOX5-mediated ferroptosis acts as a distinct cell death pathway upon oxidative stress in Huntington's disease

S Song, Z Su, N Kon, B Chu, H Li, X Jiang… - Genes & …, 2023 - genesdev.cshlp.org
Although it is well established that Huntington's disease (HD) is mainly caused by
polyglutamine-expanded mutant huntingtin (mHTT), the molecular mechanism of mHTT …

Protein interaction networks in neurodegenerative diseases: From physiological function to aggregation

G Calabrese, C Molzahn, T Mayor - Journal of Biological Chemistry, 2022 - ASBMB
The accumulation of protein inclusions is linked to many neurodegenerative diseases that
typically develop in older individuals, due to a combination of genetic and environmental …

Molecular pathophysiological mechanisms in Huntington's disease

A Jurcau - Biomedicines, 2022 - mdpi.com
Huntington's disease is an inherited neurodegenerative disease described 150 years ago
by George Huntington. The genetic defect was identified in 1993 to be an expanded CAG …

Mutant Huntingtin stalls ribosomes and represses protein synthesis in a cellular model of Huntington disease

M Eshraghi, PP Karunadharma, J Blin… - Nature …, 2021 - nature.com
The polyglutamine expansion of huntingtin (mHTT) causes Huntington disease (HD) and
neurodegeneration, but the mechanisms remain unclear. Here, we found that mHtt promotes …

Melatonin inhibits cytosolic mitochondrial DNA–induced neuroinflammatory signaling in accelerated aging and neurodegeneration

A Jauhari, SV Baranov, Y Suofu, J Kim… - The Journal of …, 2020 - Am Soc Clin Investig
Chronic inflammation is a pathologic feature of neurodegeneration and aging; however, the
mechanism regulating this process is not understood. Melatonin, an endogenous free …

The mitochondrial unfolded protein response: A multitasking giant in the fight against human diseases

Z Zhou, Y Fan, R Zong, K Tan - Ageing Research Reviews, 2022 - Elsevier
Mitochondria, which serve as the energy factories of cells, are involved in cell differentiation,
calcium homeostasis, amino acid and fatty acid metabolism and apoptosis. In response to …

Interaction of oxidative stress and misfolded proteins in the mechanism of neurodegeneration

AY Abramov, EV Potapova, VV Dremin, AV Dunaev - Life, 2020 - mdpi.com
Aggregation of the misfolded proteins β-amyloid, tau, huntingtin, and α-synuclein is one of
the most important steps in the pathology underlying a wide spectrum of neurodegenerative …