Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment
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 oxy...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2016-06-01
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| Series: | Data in Brief |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2352340916301469 |
| _version_ | 1818110225620664320 |
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| author | I. Bravo-Alonso A. Oyarzabal M. Sánchez-Aragó M.T. Rejas B. Merinero A. García-Cazorla R. Artuch M. Ugarte P. Rodríguez-Pombo |
| author_facet | I. Bravo-Alonso A. Oyarzabal M. Sánchez-Aragó M.T. Rejas B. Merinero A. García-Cazorla R. Artuch M. Ugarte P. Rodríguez-Pombo |
| author_sort | I. Bravo-Alonso |
| collection | DOAJ |
| description | 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. Keywords: BCKDK interference, Branched-chain α-ketoacid dehydrogenase, Bioenergetics profile, Unrestrained branched-chain amino acids catabolism |
| first_indexed | 2024-12-11T02:43:46Z |
| format | Article |
| id | doaj.art-601c86de12e84ee38ce5e1041e1ef956 |
| institution | Directory Open Access Journal |
| issn | 2352-3409 |
| language | English |
| last_indexed | 2024-12-11T02:43:46Z |
| publishDate | 2016-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Data in Brief |
| spelling | doaj.art-601c86de12e84ee38ce5e1041e1ef9562022-12-22T01:23:29ZengElsevierData in Brief2352-34092016-06-017755759Dataset reporting BCKDK interference in a BCAA-catabolism restricted environmentI. Bravo-Alonso0A. Oyarzabal1M. Sánchez-Aragó2M.T. Rejas3B. Merinero4A. García-Cazorla5R. Artuch6M. Ugarte7P. Rodríguez-Pombo8Centro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, SpainCentro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, SpainDepartamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa, (CSIC-UAM), U-713 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, Instituto de Investigación Hospital 12 de Octubre, Universidad Autónoma de Madrid, SpainServicio de Microscopía Electrónica, Centro de Biología Molecular Severo Ochoa, Consejo Superior de Investigaciones Científicas, Universidad Autónoma de Madrid, Madrid, SpainCentro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, SpainDepartment of Neurology, Hospital Sant Joan de Déu (HSJD), U-703 CIBER de Enfermedades Raras (CIBERER), Barcelona, SpainDepartment of Biochemistry, Hospital Sant Joan de Déu (HSJD), U-703 CIBER de Enfermedades Raras (CIBERER), Barcelona, SpainCentro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, SpainCentro de Diagnóstico de Enfermedades Moleculares (CEDEM), Departamento de Biología Molecular, Centro de Biología Molecular Severo Ochoa (CSIC-UAM), U-746 Centro de Investigación Biomédica en Red de Enfermedades Raras CIBERER-ISCIII, IDIPAZ, Universidad Autónoma de Madrid, Spain; Corresponding author.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. Keywords: BCKDK interference, Branched-chain α-ketoacid dehydrogenase, Bioenergetics profile, Unrestrained branched-chain amino acids catabolismhttp://www.sciencedirect.com/science/article/pii/S2352340916301469 |
| spellingShingle | I. Bravo-Alonso A. Oyarzabal M. Sánchez-Aragó M.T. Rejas B. Merinero A. García-Cazorla R. Artuch M. Ugarte P. Rodríguez-Pombo Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment Data in Brief |
| title | Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment |
| title_full | Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment |
| title_fullStr | Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment |
| title_full_unstemmed | Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment |
| title_short | Dataset reporting BCKDK interference in a BCAA-catabolism restricted environment |
| title_sort | dataset reporting bckdk interference in a bcaa catabolism restricted environment |
| url | http://www.sciencedirect.com/science/article/pii/S2352340916301469 |
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