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http://hdl.handle.net/2445/179115
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DC Field | Value | Language |
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dc.contributor.author | González Casacuberta, Ingrid | - |
dc.contributor.author | Juárez Flores, Diana Luz | - |
dc.contributor.author | Ezquerra, Mario | - |
dc.contributor.author | Fucho Salvador, Raquel | - |
dc.contributor.author | Catalán García, Marc | - |
dc.contributor.author | Guitart Mampel, Mariona | - |
dc.contributor.author | Tobías, Ester | - |
dc.contributor.author | García Ruiz, Carmen | - |
dc.contributor.author | Fernández Santiago, Rubén | - |
dc.contributor.author | Tolosa, Eduardo | - |
dc.contributor.author | Martí Domènech, Ma. Josep | - |
dc.contributor.author | Grau Junyent, Josep M. (Josep Maria) | - |
dc.contributor.author | Fernández Checa Torres, José Carlos | - |
dc.contributor.author | Cardellach, Francesc | - |
dc.contributor.author | Morén Núñez, Constanza | - |
dc.contributor.author | Garrabou Tornos, Glòria | - |
dc.date.accessioned | 2021-07-15T14:40:22Z | - |
dc.date.available | 2021-07-15T14:40:22Z | - |
dc.date.issued | 2019-06-09 | - |
dc.identifier.issn | 1945-4589 | - |
dc.identifier.uri | http://hdl.handle.net/2445/179115 | - |
dc.description.abstract | PRKN encodes an E3-ubiquitin-ligase involved in multiple cell processes including mitochondrial homeostasis and autophagy. Previous studies reported alterations of mitochondrial function in fibroblasts from patients with PRKN mutation-associated Parkinson's disease (PRKN-PD) but have been only conducted in glycolytic conditions, potentially masking mitochondrial alterations. Additionally, autophagy flux studies in this cell model are missing.We analyzed mitochondrial function and autophagy in PRKN-PD skin-fibroblasts (n=7) and controls (n=13) in standard (glucose) and mitochondrial-challenging (galactose) conditions.In glucose, PRKN-PD fibroblasts showed preserved mitochondrial bioenergetics with trends to abnormally enhanced mitochondrial respiration that, accompanied by decreased CI, may account for the increased oxidative stress. In galactose, PRKN-PD fibroblasts exhibited decreased basal/maximal respiration vs. controls and reduced mitochondrial CIV and oxidative stress compared to glucose, suggesting an inefficient mitochondrial oxidative capacity to meet an extra metabolic requirement. PRKN-PD fibroblasts presented decreased autophagic flux with reduction of autophagy substrate and autophagosome synthesis in both conditions.The alterations exhibited under neuron-like oxidative environment (galactose), may be relevant to the disease pathogenesis potentially explaining the increased susceptibility of dopaminergic neurons to undergo degeneration. Abnormal PRKN-PD phenotype supports the usefulness of fibroblasts to model disease and the view of PD as a systemic disease where molecular alterations are present in peripheral tissues. | - |
dc.format.extent | 18 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Impact Journals | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.18632/aging.102014 | - |
dc.relation.ispartof | Aging, 2019, vol. 11, num. 11, p. 3750-3767 | - |
dc.relation.uri | https://doi.org/10.18632/aging.102014 | - |
dc.rights | cc-by (c) González Casacuberta, Ingrid et al., 2019 | - |
dc.rights.uri | https://creativecommons.org/licenses/by/4.0/ | - |
dc.source | Articles publicats en revistes (Medicina) | - |
dc.subject.classification | Malaltia de Parkinson | - |
dc.subject.classification | Pell | - |
dc.subject.other | Parkinson's disease | - |
dc.subject.other | Skin | - |
dc.title | Mitochondrial and autophagic alterations in skin fibroblasts from Parkinson disease patients with Parkin mutations. | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 693577 | - |
dc.date.updated | 2021-07-15T14:40:23Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 31180333 | - |
Appears in Collections: | Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer) Articles publicats en revistes (Medicina) |
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File | Description | Size | Format | |
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693577.pdf | 1.22 MB | Adobe PDF | View/Open |
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