A systematic review of transcriptional dysregulation in Huntington's disease studied by RNA sequencing

B Malla, X Guo, G Senger, Z Chasapopoulou… - Frontiers in …, 2021 - frontiersin.org
Huntington's disease (HD) is a chronic neurodegenerative disorder caused by an expansion
of polyglutamine repeats in exon 1 of the Huntingtin gene. Transcriptional dysregulation …

Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure

JD Panes, A Wendt, O Ramirez-Molina… - Neural regeneration …, 2022 - journals.lww.com
The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The
main features of neurodegenerative diseases have been related with cellular and molecular …

A glimpse of molecular biomarkers in Huntington's disease

S Martí-Martínez, LM Valor - International journal of molecular sciences, 2022 - mdpi.com
Huntington's disease (HD) is a devastating neurodegenerative disorder that is caused by an
abnormal expansion of CAG repeats in the Huntingtin (HTT) gene. Although the main …

Neuron type‐specific increase in lamin B1 contributes to nuclear dysfunction in Huntington's disease

R Alcalá‐Vida, M Garcia‐Forn… - EMBO Molecular …, 2021 - embopress.org
Lamins are crucial proteins for nuclear functionality. Here, we provide new evidence
showing that increased lamin B1 levels contribute to the pathophysiology of Huntington's …

DNA methylation in huntington's disease

N Zsindely, F Siági, L Bodai - International Journal of Molecular Sciences, 2021 - mdpi.com
Methylation of cytosine in CpG dinucleotides is the major DNA modification in mammalian
cells that is a key component of stable epigenetic marks. This modification, which on the one …

[HTML][HTML] Cell-autonomous and non-cell-autonomous pathogenic mechanisms in Huntington's disease: insights from in vitro and in vivo models

J Creus-Muncunill, ME Ehrlich - Neurotherapeutics, 2019 - Elsevier
Huntington's disease (HD) is an autosomal dominant disorder caused by an expansion in
the trinucleotide CAG repeat in exon-1 in the huntingtin gene, located on chromosome 4 …

D1R-and D2R-medium-sized spiny neurons diversity: insights into striatal vulnerability to Huntington's disease mutation

G Bergonzoni, J Döring, M Biagioli - Frontiers in Cellular …, 2021 - frontiersin.org
Huntington's disease (HD) is a devastating neurodegenerative disorder caused by an
aberrant expansion of the CAG tract within the exon 1 of the HD gene, HTT. HD …

Retinal dysfunction in Huntington's disease mouse models concurs with local gliosis and microglia activation

F Cano-Cano, F Martín-Loro, A Gallardo-Orihuela… - Scientific Reports, 2024 - nature.com
Huntington's disease (HD) is caused by an aberrant expansion of CAG repeats in the HTT
gene that mainly affects basal ganglia. Although striatal dysfunction has been widely studied …

Epigenetic regulation in Huntington's disease

JW Hyeon, AH Kim, H Yano - Neurochemistry international, 2021 - Elsevier
Huntington's disease (HD) is a devastating and fatal monogenic neurodegenerative disorder
characterized by progressive loss of selective neurons in the brain and is caused by an …

[HTML][HTML] Environmental stimulation in Huntington disease patients and animal models

A Novati, HP Nguyen, J Schulze-Hentrich - Neurobiology of Disease, 2022 - Elsevier
While Huntington disease (HD) is caused solely by a polyglutamine expansion in the
huntingtin gene, environmental factors can influence HD onset and progression. Here, we …