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http://hdl.handle.net/10261/151205
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Campo DC | Valor | Lengua/Idioma |
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dc.contributor.author | Bravo-Alonso, Irene | - |
dc.contributor.author | Oyarzábal, Alfonso | - |
dc.contributor.author | Sánchez-Aragó, María | - |
dc.contributor.author | Rejas, M. Teresa | - |
dc.contributor.author | Merinero, Begoña | - |
dc.contributor.author | García-Cazorla, Angels | - |
dc.contributor.author | Artuch, Rafael | - |
dc.contributor.author | Ugarte, Magdalena | - |
dc.contributor.author | Rodríguez-Pombo, Pilar | - |
dc.date.accessioned | 2017-06-12T08:54:56Z | - |
dc.date.available | 2017-06-12T08:54:56Z | - |
dc.date.issued | 2016-03-15 | - |
dc.identifier | doi: 10.1016/j.dib.2016.03.038 | - |
dc.identifier | issn: 2352-3409 | - |
dc.identifier.citation | Data in Brief 7: 755- 759 (2016) | - |
dc.identifier.uri | http://hdl.handle.net/10261/151205 | - |
dc.description.abstract | This data article contains complementary figures to the research article >Mitochondrial response to the BCKDK-deficiency: some clues to understand the positive dietary response in this form of autism> [. 1]. Herein we present data relative to the effect of knocking down BCKDK gene on the real time oxygen consumption rate of fibroblasts obtained from a Maple Syrup Urine Disease (MSUD) patient. Interference of BCKDK expression on such cells showing a reduced branched-chain α-ketoacid dehydrogenase (BCKDHc) activity; let us generate a scenario to study the direct effect of BCKDK absence in an environment of high branched-chain amino acids (BCAAs) concentrations. Data relative to the effectiveness of the knockdown together with the potentiality of the BCKDK-knockdown to increase the deficient branched-chain α-ketoacid dehydrogenase activity detected in MSUD patients are also shown. | - |
dc.description.sponsorship | Centro de Diagnóstico de Enfermedades Moleculares (CEDEM). Irene Bravo-Alonso is Granted from Funda- ción Ramón Areces (CIVP16A1853) | - |
dc.relation.isversionof | Publisher's version | - |
dc.rights | openAccess | - |
dc.subject | BCKDK interference | - |
dc.subject | Bioenergetics profile Unrestrainedbranched-chainaminoacids catabolism | - |
dc.subject | Branched-chain α-ketoacid dehydrogenase | - |
dc.title | Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment | - |
dc.type | artículo | - |
dc.identifier.doi | 10.1016/j.dib.2016.03.038 | - |
dc.date.updated | 2017-06-12T08:54:56Z | - |
dc.description.version | Peer Reviewed | - |
dc.language.rfc3066 | eng | - |
dc.rights.license | https://creativecommons.org/licenses/by/4.0/ | - |
dc.contributor.funder | Centro de Diagnóstico de Enfermedades Moleculares (España) | - |
dc.contributor.funder | Fundación Ramón Areces | - |
dc.relation.csic | Sí | - |
dc.identifier.funder | http://dx.doi.org/10.13039/100008054 | es_ES |
dc.identifier.pmid | 27054191 | - |
dc.type.coar | http://purl.org/coar/resource_type/c_6501 | es_ES |
item.cerifentitytype | Publications | - |
item.grantfulltext | open | - |
item.openairecristype | http://purl.org/coar/resource_type/c_18cf | - |
item.fulltext | With Fulltext | - |
item.openairetype | artículo | - |
Aparece en las colecciones: | (CBM) Artículos |
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Fichero | Descripción | Tamaño | Formato | |
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RejasMT_DatasetReportingBCKDKInterferenceInaBCAA.pdf | 386,73 kB | Adobe PDF | Visualizar/Abrir |
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