Mitophagy in Alzheimer's disease and other age-related neurodegenerative diseases

Q Cai, YY Jeong - Cells, 2020 - mdpi.com
Mitochondrial dysfunction is a central aspect of aging and neurodegenerative diseases,
including Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, and …

Microglial activation in the pathogenesis of Huntington's disease

HM Yang, S Yang, SS Huang, BS Tang… - Frontiers in aging …, 2017 - frontiersin.org
Huntington's disease (HD) is an autosomal dominantly inherited neurodegenerative
disorder caused by expanded CAG trinucleotide repeats (> 36) in exon 1 of HTT gene that …

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 …

Pathologic polyglutamine aggregation begins with a self-poisoning polymer crystal

T Kandola, S Venkatesan, J Zhang, BT Lerbakken… - Elife, 2023 - elifesciences.org
A long-standing goal of amyloid research has been to characterize the structural basis of the
rate-determining nucleating event. However, the ephemeral nature of nucleation has made …

Reinstating aberrant mTORC1 activity in Huntington's disease mice improves disease phenotypes

JH Lee, L Tecedor, YH Chen, AM Monteys, MJ Sowada… - Neuron, 2015 - cell.com
Huntington's disease (HD) is caused by a polyglutamine tract expansion in huntingtin (HTT).
Despite HTTs ubiquitous expression, there is early and robust vulnerability in striatum, the …

Mfn2 is required for mitochondrial development and synapse formation in human induced pluripotent stem cells/hiPSC derived cortical neurons

D Fang, S Yan, Q Yu, D Chen, SSD Yan - Scientific reports, 2016 - nature.com
Mitochondria are essential dynamic organelles for energy production. Mitochondria
dynamically change their shapes tightly coupled to fission and fusion. Imbalance of fission …

Rhes, a striatal-selective protein implicated in Huntington disease, binds beclin-1 and activates autophagy

RG Mealer, AJ Murray, N Shahani… - Journal of Biological …, 2014 - ASBMB
The protein mutated in Huntington disease (HD), mutant huntingtin (mHtt), is expressed
throughout the brain and body. However, the pathology of HD is characterized by early and …

Huntington disease as a neurodevelopmental disorder and early signs of the disease in stem cells

K Wiatr, WJ Szlachcic, M Trzeciak, M Figlerowicz… - Molecular …, 2018 - Springer
Huntington disease (HD) is a dominantly inherited disorder caused by a CAG expansion
mutation in the huntingtin (HTT) gene, which results in the HTT protein that contains an …

Transcription, epigenetics and ameliorative strategies in Huntington's Disease: a genome-wide perspective

LM Valor - Molecular neurobiology, 2015 - Springer
Transcriptional dysregulation in Huntington's disease (HD) is an early event that shapes the
brain transcriptome by both the depletion and ectopic activation of gene products that …

Huntingtin promotes mTORC1 signaling in the pathogenesis of Huntington's disease

WM Pryor, M Biagioli, N Shahani, S Swarnkar… - Science …, 2014 - science.org
In patients with Huntington's disease (HD), the protein huntingtin (Htt) has an expanded
polyglutamine (poly-Q) tract. HD results in early loss of medium spiny neurons in the …