Mitochondrial signalling and homeostasis: from cell biology to neurological disease
JJ Collier, M Oláhová, TG McWilliams… - Trends in …, 2023 - cell.com
Efforts to understand how mitochondrial dysfunction contributes to neurodegeneration have
primarily focussed on the role of mitochondria in neuronal energy metabolism. However …
primarily focussed on the role of mitochondria in neuronal energy metabolism. However …
Antisense oligonucleotide therapy: from design to the Huntington disease clinic
ME Rook, AL Southwell - BioDrugs, 2022 - Springer
Huntington disease (HD) is a fatal progressive neurodegenerative disorder caused by an
inherited mutation in the huntingtin (HTT) gene, which encodes mutant HTT protein. Though …
inherited mutation in the huntingtin (HTT) gene, which encodes mutant HTT protein. Though …
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 …
polyglutamine of huntingtin (mHTT). mHTT is ubiquitously present, but it induces noticeable …
Hypothalamic alterations in neurodegenerative diseases and their relation to abnormal energy metabolism
P Vercruysse, D Vieau, D Blum, Å Petersén… - Frontiers in molecular …, 2018 - frontiersin.org
Neurodegenerative diseases (NDDs) are disorders characterized by progressive
deterioration of brain structure and function. Selective neuronal populations are affected …
deterioration of brain structure and function. Selective neuronal populations are affected …
Alterations in synaptic function and plasticity in Huntington disease
AI Smith‐Dijak, MD Sepers… - Journal of …, 2019 - Wiley Online Library
Huntington disease (HD) is an inherited neurodegenerative disorder caused by an
expansion of the CAG repeat region in the first exon of the huntingtin gene …
expansion of the CAG repeat region in the first exon of the huntingtin gene …
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 …
disorder caused by expanded CAG trinucleotide repeats (> 36) in exon 1 of HTT gene that …
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 …
ubiquitously expressed gene, yet it prominently damages the striatum and cortex, followed …
The translational potential of cholesterol-based therapies for neurological disease
M Valenza, G Birolini, E Cattaneo - Nature Reviews Neurology, 2023 - nature.com
Cholesterol is an important metabolite and membrane component and is enriched in the
brain owing to its role in neuronal maturation and function. In the adult brain, cholesterol is …
brain owing to its role in neuronal maturation and function. In the adult brain, cholesterol is …
A novel Huntington's disease assessment platform to support future drug discovery and development
J Wu, L Möhle, T Brüning, I Eiriz, M Rafehi… - International Journal of …, 2022 - mdpi.com
Huntington's disease (HD) is a lethal neurodegenerative disorder without efficient
therapeutic options. The inefficient translation from preclinical and clinical research into …
therapeutic options. The inefficient translation from preclinical and clinical research into …
AAV5-miHTT gene therapy demonstrates sustained huntingtin lowering and functional improvement in Huntington disease mouse models
EA Spronck, CC Brouwers, A Vallès, M de Haan… - … Therapy-Methods & …, 2019 - cell.com
Huntington disease (HD) is a fatal neurodegenerative disorder caused by an autosomal
dominant CAG repeat expansion in the huntingtin (HTT) gene. The translated expanded …
dominant CAG repeat expansion in the huntingtin (HTT) gene. The translated expanded …