Mitochondrial abnormalities and synaptic damage in Huntington's disease: a focus on defective mitophagy and mitochondria-targeted therapeutics

N Sawant, H Morton, S Kshirsagar, AP Reddy… - Molecular …, 2021 - Springer
Huntington's disease (HD) is a fatal and pure genetic disease with a progressive loss of
medium spiny neurons (MSN). HD is caused by expanded polyglutamine repeats in the …

[HTML][HTML] Targeting the proteostasis network in Huntington's disease

TR Soares, SD Reis, BR Pinho, MR Duchen… - Ageing Research …, 2019 - Elsevier
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by
a polyglutamine expansion mutation in the huntingtin protein. Expansions above 40 …

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 …

Cyclic GMP-AMP synthase promotes the inflammatory and autophagy responses in Huntington disease

M Sharma, S Rajendrarao, N Shahani… - Proceedings of the …, 2020 - National Acad Sciences
Huntington disease (HD) is caused by an expansion mutation of the N-terminal
polyglutamine of huntingtin (mHTT). mHTT is ubiquitously present, but it induces noticeable …

Deletion of SUMO1 attenuates behavioral and anatomical deficits by regulating autophagic activities in Huntington disease

UN Ramírez-Jarquín, M Sharma… - Proceedings of the …, 2022 - National Acad Sciences
The CAG expansion of huntingtin (mHTT) associated with Huntington disease (HD) is a
ubiquitously expressed gene, yet it prominently damages the striatum and cortex, followed …

Rhes travels from cell to cell and transports Huntington disease protein via TNT-like protrusion

M Sharma, S Subramaniam - Journal of Cell Biology, 2019 - rupress.org
Tunneling nanotubes (TNT) are thin, membranous, tunnel-like cell-to-cell connections, but
the mechanisms underlying their biogenesis or functional role remains obscure. Here, we …

[HTML][HTML] Focus: Death: Selective Neuronal Death in Neurodegenerative Diseases: The Ongoing Mystery

S Subramaniam - The Yale journal of biology and medicine, 2019 - ncbi.nlm.nih.gov
A major unresolved problem in neurodegenerative disease is why and how a specific set of
neurons in the brain are highly vulnerable to neuronal death. Multiple pathways and …

Loss of Hap1 selectively promotes striatal degeneration in Huntington disease mice

Q Liu, S Cheng, H Yang, L Zhu, Y Pan… - Proceedings of the …, 2020 - National Acad Sciences
Huntington disease (HD) is an ideal model for investigating selective neurodegeneration, as
expanded polyQ repeats in the ubiquitously expressed huntingtin (HTT) cause the …

Selective Neuron Vulnerability in Common and Rare Diseases—Mitochondria in the Focus

T Paß, RJ Wiesner, D Pla-Martín - Frontiers in molecular biosciences, 2021 - frontiersin.org
Mitochondrial dysfunction is a central feature of neurodegeneration within the central and
peripheral nervous system, highlighting a strong dependence on proper mitochondrial …

Rhes, a striatal-enriched protein, promotes mitophagy via Nix

M Sharma, UNR Jarquin, O Rivera… - Proceedings of the …, 2019 - National Acad Sciences
Elimination of dysfunctional mitochondria via mitophagy is essential for cell survival and
neuronal functions. But, how impaired mitophagy participates in tissue-specific vulnerability …