I04 Reduction of glutamate dehydrogenase increases autophagy and ameliorate motility and survival in a drosophila model for huntington’s disease

2021 ◽  
Author(s):  
Paola Bellosta ◽  
Stefania Santarelli ◽  
Chiara Londero
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Glutamate Dehydrogenase ◽  
Drosophila Model

Methylene Blue Partially Rescues Heart Defects in a Drosophila Model of Huntington’s Disease

2015 ◽  
Vol 4 (2) ◽  
pp. 173-186 ◽  
Author(s):  
Raheleh Heidari ◽  
Véronique Monnier ◽  
Elodie Martin ◽  
Hervé Tricoire
Keyword(s):  
Methylene Blue ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Heart Defects ◽  
Drosophila Model

NMR Spectroscopy-based Metabolomics of Drosophila Model of Huntington’s Disease Suggests Altered Cell Energetics

2017 ◽  
Vol 16 (10) ◽  
pp. 3863-3872 ◽  
Author(s):  
Virender Singh ◽  
Raj Kumar Sharma ◽  
Thamarailingam Athilingam ◽  
Pradip Sinha ◽  
Neeraj Sinha ◽  
...  
Keyword(s):  
Nmr Spectroscopy ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Drosophila Model ◽  
Altered Cell

scholarly journals Esculetin Provides Neuroprotection against Mutant Huntingtin-Induced Toxicity in Huntington’s Disease Models

2021 ◽  
Vol 14 (10) ◽  
pp. 1044
Author(s):  
Letizia Pruccoli ◽  
Carlo Breda ◽  
Gabriella Teti ◽  
Mirella Falconi ◽  
Flaviano Giorgini ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuronal Death ◽  
Trinucleotide Repeat ◽  
Neurodegenerative Disorder ◽  
Positive Impact ◽  
Neuroprotective Effects ◽  
Drosophila Model ◽  
Exon 1 ◽  
Transgenic Drosophila

Huntington’s disease (HD) is a neurodegenerative disorder caused by an abnormal CAG trinucleotide repeat expansion within exon 1 of the huntingtin (HTT) gene. This mutation leads to the production of mutant HTT (mHTT) protein which triggers neuronal death through several mechanisms. Here, we investigated the neuroprotective effects of esculetin (ESC), a bioactive phenolic compound, in an inducible PC12 model and a transgenic Drosophila melanogaster model of HD, both of which express mHTT fragments. ESC partially inhibited the progression of mHTT aggregation and reduced neuronal death through its ability to counteract the oxidative stress and mitochondria impairment elicited by mHTT in the PC12 model. The ability of ESC to counteract neuronal death was also confirmed in the transgenic Drosophila model. Although ESC did not modify the lifespan of the transgenic Drosophila, it still seemed to have a positive impact on the HD phenotype of this model. Based on our findings, ESC may be further studied as a potential neuroprotective agent in a rodent transgenic model of HD.

scholarly journals Increased energy metabolism rescues glia-induced pathology in a Drosophila model of Huntington's disease

2010 ◽  
Vol 19 (17) ◽  
pp. 3372-3382 ◽  
Author(s):  
Marie-Thérèse Besson ◽  
Pascale Dupont ◽  
Yih-Woei C. Fridell ◽  
Jean-Charles Liévens
Keyword(s):  
Energy Metabolism ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Drosophila Model

A38 Mitochondrial interventions rescue disease phenotypes in a drosophila model of huntington’s disease

2018 ◽  
Author(s):  
Susanna Campesan ◽  
Kyriaki Marcou ◽  
Anna Straatman-Iwanowska ◽  
Victoria E Cotton ◽  
Charalambos P Kyriacou ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Phenotypes ◽  
Drosophila Model

AUTEN-67 (Autophagy Enhancer-67) Hampers the Progression of Neurodegenerative Symptoms in a Drosophila model of Huntington’s Disease

2016 ◽  
Vol 5 (2) ◽  
pp. 133-147 ◽  
Author(s):  
Viktor Billes ◽  
Tibor Kovács ◽  
Bernadette Hotzi ◽  
Anna Manzéger ◽  
Kinga Tagscherer ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Drosophila Model

B35 Glutamine synthetase-1 induces autophagy and neuronal survival in a drosophila model huntington’s disease

2016 ◽  
Vol 87 (Suppl 1) ◽  
pp. A21.2-A21 ◽  
Author(s):  
Maria Enrica Pasini ◽  
Chiara Paiardi ◽  
Luisa Vernizzi ◽  
Alice Valenza ◽  
Giusimaria Licata ◽  
...  
Keyword(s):  
Glutamine Synthetase ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuronal Survival ◽  
Drosophila Model

Presynaptic dysfunction in Huntington's disease

2010 ◽  
Vol 38 (2) ◽  
pp. 488-492 ◽  
Author(s):  
José L. Rozas ◽  
Leonardo Gómez-Sánchez ◽  
Cristina Tomás-Zapico ◽  
José J. Lucas ◽  
Rafael Fernández-Chacón
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Molecular Mechanisms ◽  
Neuronal Damage ◽  
Motor Dysfunction ◽  
Drosophila Model ◽  
Glutamine Repeat ◽  
Mutant Forms ◽  
The Relationship

HD (Huntington's disease) is produced by the expression of mutant forms of the protein htt (huntingtin) containing a pathologically expanded poly-glutamine repeat. For unknown reasons, in HD patients and HD mouse models, neurons from the striatum and cerebral cortex degenerate and lead to motor dysfunction and dementia. Synaptic transmission in those neurons becomes progressively altered during the course of the disease. However, the relationship between synaptic dysfunction and neurodegeneration in HD is not yet clear. Are there early specific functional synaptic changes preceding symptoms and neurodegeneration? What is the role of those changes in neuronal damage? Recent experiments in a Drosophila model of HD have showed that abnormally increased neurotransmitter release might be a leading cause of neurodegeneration. In the present review, we summarize recently described synaptic alterations in HD animal models and discuss potential underlying molecular mechanisms.

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