4 documents found, page 1 of 1
Neuroprotection of retinal ganglion cells by the sigma-1 receptor agonist prido...
Geva, Michal; Gershoni-Emek, Noga; Naia, Luana; Ly, Philip; Mota, Sandra; Rego, Ana Cristina; Hayden, Michael R.; Levin, Leonard A.Optic neuropathies such as glaucoma are characterized by retinal ganglion cell (RGC) degeneration and death. The sigma-1 receptor (S1R) is an attractive target for treating optic neuropathies as it is highly expressed in RGCs, and its absence causes retinal degeneration. Activation of the S1R exerts neuroprotective effects in models of retinal degeneration. Pridopidine is a highly selective and potent S1R agoni...
Histone Deacetylase Inhibitors Protect Against Pyruvate Dehydrogenase Dysfuncti...
Naia, Luana; Cunha-Oliveira, Teresa; Rodrigues, Joana; Rosenstock, Tatiana R.; Oliveira, Ana; Ribeiro, Márcio; Carmo, Catarina; Oliveira-Sousa, Sofia I.Transcriptional deregulation and changes in mitochondrial bioenergetics, including pyruvate dehydrogenase (PDH) dysfunction, have been described in Huntington's disease (HD). We showed previously that the histone deacetylase inhibitors (HDACIs) trichostatin A and sodium butyrate (SB) ameliorate mitochondrial function in cells expressing mutant huntingtin. In this work, we investigated the effect of HDACIs on th...
Systematic interaction network filtering identifies CRMP1 as a novel suppressor...
Stroedicke, Martin; Bounab, Yacine; Strempel, Nadine; Klockmeier, Konrad; Yigit, Sargon; Friedrich, Ralf P.; Chaurasia, Gautam; Li, ShuangAssemblies of huntingtin (HTT) fragments with expanded polyglutamine (polyQ) tracts are a pathological hallmark of Huntington's disease (HD). The molecular mechanisms by which these structures are formed and cause neuronal dysfunction and toxicity are poorly understood. Here, we utilized available gene expression data sets of selected brain regions of HD patients and controls for systematic interaction network ...
Long-term lentiviral-mediated expression of ciliary neurotrophic factor in the ...
Zala, Diana; Bensadoun, Jean-Charles; Pereira de Almeida, Luis; Leavitt, Blair R.; Gutekunst, Claire-Anne; Aebischer, Patrick; Hayden, Michael R.Ciliary neurotrophic factor (CNTF) has been shown to prevent behavioral deficits and striatal degeneration in neurotoxic models of Huntington's disease (HD), but its effect in a genetic model has not been evaluated. Lentiviral vectors expressing the human CNTF or LacZ reporter gene were therefore injected in the striatum of wild-type (WT) and transgenic mice expressing full-length huntingtin with 72 CAG repeats...