[HTML][HTML] Mitochondrial impairment: A common motif in neuropsychiatric presentation? The link to the tryptophan–kynurenine metabolic system

M Tanaka, Á Szabó, E Spekker, H Polyák, F Tóth… - Cells, 2022 - mdpi.com
Nearly half a century has passed since the discovery of cytoplasmic inheritance of human
chloramphenicol resistance. The inheritance was then revealed to take place maternally by …

Molecular mechanisms underlying nucleotide repeat expansion disorders

I Malik, CP Kelley, ET Wang, PK Todd - Nature reviews Molecular cell …, 2021 - nature.com
The human genome contains over one million short tandem repeats. Expansion of a subset
of these repeat tracts underlies over fifty human disorders, including common genetic …

[HTML][HTML] Microglia and complement mediate early corticostriatal synapse loss and cognitive dysfunction in Huntington's disease

DK Wilton, K Mastro, MD Heller, FW Gergits… - Nature medicine, 2023 - nature.com
Huntington's disease (HD) is a devastating monogenic neurodegenerative disease
characterized by early, selective pathology in the basal ganglia despite the ubiquitous …

[HTML][HTML] 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 …

[HTML][HTML] An orally available, brain penetrant, small molecule lowers huntingtin levels by enhancing pseudoexon inclusion

CG Keller, Y Shin, AM Monteys, N Renaud… - Nature …, 2022 - nature.com
Huntington's Disease (HD) is a progressive neurodegenerative disorder caused by CAG
trinucleotide repeat expansions in exon 1 of the huntingtin (HTT) gene. The mutant HTT …

[HTML][HTML] A huntingtin knockin pig model recapitulates features of selective neurodegeneration in Huntington's disease

S Yan, Z Tu, Z Liu, N Fan, H Yang, S Yang, W Yang… - Cell, 2018 - cell.com
Huntington's disease (HD) is characterized by preferential loss of the medium spiny neurons
in the striatum. Using CRISPR/Cas9 and somatic nuclear transfer technology, we …

[HTML][HTML] Elusive roles for reactive astrocytes in neurodegenerative diseases

L Ben Haim, MA Carrillo-de Sauvage… - Frontiers in cellular …, 2015 - frontiersin.org
Astrocytes play crucial roles in the brain and are involved in the neuroinflammatory
response. They become reactive in response to virtually all pathological situations in the …

[HTML][HTML] New avenues for the treatment of Huntington's disease

A Kim, K Lalonde, A Truesdell, P Gomes Welter… - International journal of …, 2021 - mdpi.com
Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG expansion in
the HD gene. The disease is characterized by neurodegeneration, particularly in the striatum …

ALOX5-mediated ferroptosis acts as a distinct cell death pathway upon oxidative stress in Huntington's disease

S Song, Z Su, N Kon, B Chu, H Li, X Jiang… - Genes & …, 2023 - genesdev.cshlp.org
Although it is well established that Huntington's disease (HD) is mainly caused by
polyglutamine-expanded mutant huntingtin (mHTT), the molecular mechanism of mHTT …

[HTML][HTML] RAN translation in Huntington disease

M Banez-Coronel, F Ayhan, AD Tarabochia, T Zu… - Neuron, 2015 - cell.com
Huntington disease (HD) is caused by a CAG⋅ CTG expansion in the huntingtin (HTT) gene.
While most research has focused on the HTT polyGln-expansion protein, we demonstrate …