Výsledky vyhledávání - "Huntington Disease/metabolism"

Therapeutic efficacy of regulable GDNF expression for Huntington's and Parkinson's disease by a high-induction, background-free 'GeneSwitch' vector

Autor: Virginie Zimmer, Sebastian Kügler, Shi Cheng, Frank Streit, Catherine Pythoud, Jolanda M. Liefhebber, Anupam Raina, Pavlina Konstantinova, Nicole Déglon, Maria Rey, Gabriel Vachey, Julia Tereshchenko, Andrzej Mazur, Mathias Bähr

Publikováno v: Experimental neurology, vol. 309, pp. 79-90

Gene therapy is currently an irreversible approach, without possibilities to fine-tune or halt the expression of a therapeutic gene product. Especially when expressing neurotrophic factors to treat neurodegenerative disorders, options to regulate tra

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ec6bc910cb868b3ba3bfa0229d4aa882
https://doi.org/10.1016/j.expneurol.2018.07.017

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Cell-Type-Specific Gene Expression Profiling in Adult Mouse Brain Reveals Normal and Disease-State Signatures

Autor: Merienne, Nicolas, Meunier, Cécile, Schneider, Anne, Seguin, Jonathan, Nair, Satish, Rocher, Anne, Le Gras, Stéphanie, Keime, Celine, Faull, Richard, Pellerin, Luc, Chatton, Jean-Yves, Neri, Christian, Mérienne, Karine, Déglon, Nicole

Publikováno v: Cell Reports
Cell Reports, Elsevier Inc, 2019, 26 (9), pp.2477-2493.e9. ⟨10.1016/j.celrep.2019.02.003⟩
Cell reports, vol. 26, no. 9, pp. 2477-2493.e9

International audience; The role of brain cell-type-specific functions and profiles in pathological and non-pathological contexts is still poorly defined. Such cell-type-specific gene expression profiles in solid, adult tissues would benefit from app

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::06c1cb01d438461d088ec232312a83d1
https://hal.sorbonne-universite.fr/hal-02087719

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Downstream mechanisms triggered by mitochondrial dysfunction in the basal ganglia: From experimental models to neurodegenerative diseases

Autor: Paolo Gubellini, Barbara Picconi, Massimiliano Di Filippo, Paolo Calabresi

Publikováno v: Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease; Vol 1802
Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease
Biochimica et Biophysica Acta-Molecular Basis of Disease
Biochimica et Biophysica Acta-Molecular Basis of Disease, Elsevier, 2009, 1802 (1), pp.151. ⟨10.1016/j.bbadis.2009.08.001⟩
BBA-Biochimica et Biophysica Acta
BBA-Biochimica et Biophysica Acta, Elsevier, 2010, 1802 (1), pp.151-61. ⟨10.1016/j.bbadis.2009.08.001⟩
BBA-Biochimica et Biophysica Acta, 2010, 1802 (1), pp.151-61. ⟨10.1016/j.bbadis.2009.08.001⟩

International audience; Mitochondrial dysfunctions have been implicated in the cellular processes underlying several neurodegenerative disorders affecting the basal ganglia. These include Huntington's chorea and Parkinson's disease, two highly debili

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2cff29678effa65637604fb0efb2c08b

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CA150 expression delays striatal cell death in overexpression and knock-in conditions for mutant huntingtin neurotoxicity

Autor: Ashwani Kumar Thakur, Emmanuel Brouillet, Christian Neri, James Pearson, Patrick Aebischer, Margarita Arango, Sébastien Holbert, Etienne Régulier, Dania Zala, Nicole Déglon, Ronald Wetzel

Publikováno v: Journal of Neuroscience
Journal of Neuroscience, 2006, 26 (17), pp.4649-4659. ⟨10.1523/JNEUROSCI.5409-05.2006⟩
Journal of Neuroscience, Society for Neuroscience, 2006, 26 (17), pp.4649-4659. ⟨10.1523/JNEUROSCI.5409-05.2006⟩

Transcriptional dysregulation caused by expanded polyglutamines (polyGlns) in huntingtin (htt) may be central to cell-autonomous mechanisms for neuronal cell death in Huntington's disease (HD) pathogenesis. We hypothesized that these mechanisms may i

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c07f4e24a47f4a28df9ca507139174fb
https://hal.inrae.fr/hal-02666883

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Insulin-like growth factor-I receptor densities in human frontal cortex and white matter during aging, in Alzheimer's disease, and in Huntington's disease

Autor: Jacques De Keyser, Anita Goossens, N. Wilczak

Using quantitative autoradiography we have investigated insulin-like growth factor (IGF)-I receptors in postmortem-obtained frontal cortex and white matter from 39 individuals without neurological disease, ranging in age from 0 to 95 years, and from

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b947c3adbd4d8ed94ca11a3d0deeb1a8
https://hdl.handle.net/20.500.14017/dab9bd12-967d-4703-9dec-2de603be9e5d

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Coupling of D1 dopamine receptors to the guanine nucleotide binding protein Gs is deficient in Huntington's disease

Autor: Jean-Paul De Backer, Georges Vauquelin, Jacques De Keyser, Guy Ebinger

Publikováno v: Brain research. 496(1-2)

Human brain contains two subtypes of D1 dopamine receptors, which both exist under high- (RH) and low-agonist affinity (RL) sites, but can be distinguished on the basis of the ability of GTP to convert RH into RL. The amygdala contains exclusively GT

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a6aab5b78634772d5cd07d2dd7b3065e
https://pubmed.ncbi.nlm.nih.gov/2529949

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Lack of GTP-insensitive D2 dopamine receptors in Huntington's disease

Autor: Jacques De Keyser, G. Vauquelin, Guy Ebinger, Raymund A.C. Roos

Publikováno v: Journal of the neurological sciences. 92(2-3)

Previous studies have shown that the mammalian neostriatum contains two subtypes of D2 dopamine receptors, which can be distinguished on the basis of the ability of GTP to convert high (RH) into low (RL) affinity sites for dopamine: GTP-sensitive (GS

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0215adde4f970f391ae4a316605254f5
https://pubmed.ncbi.nlm.nih.gov/2150971

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