Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
Background: Huntington's disease (HD) is a progressive neurodegenerative disorder caused by CAG repeat expansions in the HTT gene. Somatic repeat expansion in the R6/1 mouse model of HD depends on mismatch repair and is worsened by base excision repair initiated by the 7,8-dihydroxy-8-oxoguanine-DNA glycosylase (Ogg1) or Nei-like 1 (Neil1). Ogg1 and Neil1 repairs common oxidative lesions.
Methods: We investigated whether anthocyanin antioxidants added daily to the drinking water could affect CAG repeat instability in several organs and behaviour in R6/1 HD mice. In addition, anthocyanin-treated and untreated R6/1 HD mice at 22 weeks of age were tested in the open field test and on the rotarod.
Results: Anthocyanin-treated R6/1 HD mice showed reduced instability index in the ears and in the cortex compared to untreated R6/1 mice, and no difference in liver and kidney. There were no significant differences in any of the parameters tested in the behavioural tests among anthocyanin-treated and untreated R6/1 HD mice.
Conclusions: Our results indicate that continuous anthocyanin-treatment may have modest effects on CAG repeat instability in the ears and the cortex of R6/1 mice. More studies are required to investigate if anthocyanin-treatment could affect behaviour earlier in the disease course.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4973517 | PMC |
| http://dx.doi.org/10.1371/currents.hd.58d04209ab6d5de0844db7ef5628ff67 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Restoration of CB1 receptor function in hippocampal GABAergic neurons rescues memory deficits in Huntington's disease models.
Transl Neurodegener
August 2025
Departament de Biomedicina, Facultat de Medicina I Ciències de La Salut, Institut de Neurociències, Universitat de Barcelona, Barcelona, Spain.
Background: Dysregulation of the endocannabinoid system (eCBS) and the loss of CB1 receptors (CB1R) in the basal ganglia are well-established hallmarks of Huntington's disease (HD). As a result, significant research efforts have focused on targeting the eCBS to alleviate motor disturbances associated with the disease. Beyond its role in motor control, the eCBS is a complex signaling network critically involved in regulating learning and memory.
View Article and Find Full Text PDFPrebiotics improve motor function, cognition and gut health in a preclinical model of Huntington's disease.
Brain Behav Immun
August 2025
Florey Institute of Neuroscience and Mental Health, Parkville, Victoria 3052, Australia; Florey Department of Neuroscience and Mental Health, University of Melbourne, Parkville, Victoria 3052, Australia; Department of Anatomy and Physiology, University of Melbourne, Parkville, Victoria 3010, Austral
Huntington's disease (HD) is a currently incurable neurodegenerative disorder characterised by psychiatric, cognitive and motor deficits, as well as peripheral manifestations, including gastrointestinal (GI) and immunological impairments. The R6/1 mouse model of HD, expressing a mutant human huntingtin transgene, exhibits excellent construct and face validity. Evidence of gut dysbiosis has been reported in clinical and preclinical HD and is strongly associated with disease symptoms, including cognitive and behavioural outcomes.
View Article and Find Full Text PDFSertraline treatment prevents motor dysfunction in a Huntington's disease mouse model and functional decline in patients.
Neurotherapeutics
August 2025
Facultat de Medicina i Ciències de la Salut, Institut de Neurociències, Universitat de Barcelona, Barcelona, Catalonia, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain; Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegene
Molecular alterations underlying Huntington's disease (HD) are not fully elucidated and no curative therapies are available. We have described that increased translation efficiency participates in the motor symptoms in the R6/1 HD mouse model. Here, we evaluated whether sertraline, a widely used antidepressant drug, that modulates translation in cancer cells, could ameliorate motor and cognitive symptoms in HD.
View Article and Find Full Text PDFMitochondria from huntington´s disease striatal astrocytes are hypermetabolic and compromise neuronal branching.
Cell Commun Signal
July 2025
Departament de Biomedicina, Facultat de Medicina i Ciències de la Salut, Institut de Neurociències, Universitat de Barcelona, Barcelona, 08036, Spain.
Background: Deficits in mitochondrial bioenergetics and dynamics are strongly implicated in the selective vulnerability of striatal neurons in Huntington´s disease. Beyond these neuron-intrinsic factor, increasing evidence suggest that non-neuronal mechanisms, particularly astrocytic dysfunction involving disrupted homeostasis and metabolic support also contribute to disease progression. These findings underscore the critical role of metabolic crosstalk between neurons and astrocytes in maintaining striatal integrity.
View Article and Find Full Text PDFIncreased translation in adult mouse striatum is sufficient to induce motor dysfunction.
Brain Commun
June 2025
Departament de Biomedicina, Facultat de Medicina I Ciències de la Salut, Institut de Neurociències, Universitat de Barcelona, C/Casanova 143 Barcelona, Catalonia 08036, Spain.
Protein synthesis is a process finely regulated in all cell types but specially in neurons as they need rapid changes in protein concentration for synaptic plasticity. Alterations in translation rates have been shown in diseases affecting the brain. In Huntington's disease (HD), an autosomal dominant neurodegenerative disorder characterized by the presence of motor, cognitive and psychiatric symptoms, we have shown that translation is increased in the striatum contributing to motor symptoms.
View Article and Find Full Text PDF