Role of Nrf2 in aging, Alzheimer's and other neurodegenerative diseases

M George, M Tharakan, J Culberson, AP Reddy… - Ageing Research …, 2022 - Elsevier
Abstract Nuclear Factor-Erythroid Factor 2 (Nrf2) is an important transcription factor that
regulates the expression of large number of genes in healthy and disease states. Nrf2 is …

Spinocerebellar ataxias: prospects and challenges for therapy development

T Ashizawa, G Öz, HL Paulson - Nature Reviews Neurology, 2018 - nature.com
The spinocerebellar ataxias (SCAs) comprise more than 40 autosomal dominant
neurodegenerative disorders that present principally with progressive ataxia. Within the past …

[HTML][HTML] Exosome-mediated delivery of hydrophobically modified siRNA for huntingtin mRNA silencing

MC Didiot, LM Hall, AH Coles, RA Haraszti… - Molecular Therapy, 2016 - cell.com
Delivery represents a significant barrier to the clinical advancement of oligonucleotide
therapeutics for the treatment of neurological disorders, such as Huntington's disease …

Molecular mechanisms and potential therapeutical targets in Huntington's disease

C Zuccato, M Valenza, E Cattaneo - Physiological reviews, 2010 - journals.physiology.org
Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG repeat
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …

Unfolding the role of protein misfolding in neurodegenerative diseases

C Soto - Nature Reviews Neuroscience, 2003 - nature.com
Recent evidence indicates that diverse neurodegenerative diseases might have a common
cause and pathological mechanism—the misfolding, aggregation and accumulation of …

Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

C Zuccato, A Ciammola, D Rigamonti, BR Leavitt… - Science, 2001 - science.org
Huntingtin is a 350-kilodalton protein of unknown function that is mutated in Huntington's
disease (HD), a neurodegenerative disorder. The mutant protein is presumed to acquire a …

[HTML][HTML] Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules

LR Gauthier, BC Charrin, M Borrell-Pagès… - Cell, 2004 - cell.com
Polyglutamine expansion (polyQ) in the protein huntingtin is pathogenic and responsible for
the neuronal toxicity associated with Huntington's disease (HD). Although wild-type …

Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes

C Zuccato, M Tartari, A Crotti, D Goffredo, M Valenza… - Nature …, 2003 - nature.com
Huntingtin protein is mutated in Huntington disease. We previously reported that wild-type
but not mutant huntingtin stimulates transcription of the gene encoding brain-derived …

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines

AV Panov, CA Gutekunst, BR Leavitt, MR Hayden… - Nature …, 2002 - nature.com
Huntington's disease (HD) is caused by an expansion of exonic CAG triplet repeats in the
gene encoding huntingtin protein (Htt), but the mechanisms by which this mutant protein …

[HTML][HTML] The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg

A Ciechanover, P Brundin - Neuron, 2003 - cell.com
The ubiquitin-proteasome system targets numerous cellular proteins for degradation. In
addition, modifications by ubiquitin-like proteins as well as proteins containing ubiquitin …