ER stress and the unfolded protein response in neurodegeneration
The clinical manifestation of neurodegenerative diseases is initiated by the selective
alteration in the functionality of distinct neuronal populations. The pathology of many …
alteration in the functionality of distinct neuronal populations. The pathology of many …
The biology of huntingtin
F Saudou, S Humbert - Neuron, 2016 - cell.com
Huntingtin (HTT) is now a famous protein because an abnormal expansion of a glutamine
stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative …
stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative …
CRISPR/Cas9 editing of the mutant huntingtin allele in vitro and in vivo
AM Monteys, SA Ebanks, MS Keiser, BL Davidson - Molecular therapy, 2017 - cell.com
Huntington disease (HD) is a fatal dominantly inherited neurodegenerative disorder caused
by CAG repeat expansion (> 36 repeats) within the first exon of the huntingtin gene …
by CAG repeat expansion (> 36 repeats) within the first exon of the huntingtin gene …
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 …
neurodegeneration, but the mechanisms remain unclear. Here, we found that mHtt promotes …
The cryo-electron microscopy structure of huntingtin
Huntingtin (HTT) is a large (348 kDa) protein that is essential for embryonic development
and is involved in diverse cellular activities such as vesicular transport, endocytosis …
and is involved in diverse cellular activities such as vesicular transport, endocytosis …
[HTML][HTML] Salivary biomarkers for the diagnosis and monitoring of neurological diseases
Current research efforts on neurological diseases are focused on identifying novel disease
biomarkers to aid in diagnosis, provide accurate prognostic information and monitor disease …
biomarkers to aid in diagnosis, provide accurate prognostic information and monitor disease …
VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease
Mutant Huntingtin (mtHtt) causes neurodegeneration in Huntington's disease (HD) by
evoking defects in the mitochondria, but the underlying mechanisms remains elusive. Our …
evoking defects in the mitochondria, but the underlying mechanisms remains elusive. Our …
Huntingtin and the Synapse
Huntington disease (HD) is a monogenic disease that results in a combination of motor,
psychiatric and cognitive symptoms. HD is caused by a CAG trinucleotide repeat expansion …
psychiatric and cognitive symptoms. HD is caused by a CAG trinucleotide repeat expansion …
Soluble mutant huntingtin drives early human pathogenesis in Huntington's disease
A Miguez, C Gomis, C Vila… - Cellular and Molecular …, 2023 - Springer
Huntington's disease (HD) is an incurable inherited brain disorder characterised by massive
degeneration of striatal neurons, which correlates with abnormal accumulation of misfolded …
degeneration of striatal neurons, which correlates with abnormal accumulation of misfolded …
[HTML][HTML] The contribution of glial cells to Huntington's disease pathogenesis
DK Wilton, B Stevens - Neurobiology of disease, 2020 - Elsevier
Glial cells play critical roles in the normal development and function of neural circuits, but in
many neurodegenerative diseases, they become dysregulated and may contribute to the …
many neurodegenerative diseases, they become dysregulated and may contribute to the …