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Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome
Barth syndrome (BTHS) is a rare, X-linked cardiomyopathy that is characterized by abnormalities in glucose and lipid metabolism, with less known regarding amino acid metabolism. This pilot study characterized whole-body arginine kinetics and found lower arginine rate of appearance into plasma (0.69 ...
| Autores principales: | , , , , , , , , |
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
| Publicado: |
Elsevier
2020
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| Materias: | |
| Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7649643/ https://www.ncbi.nlm.nih.gov/pubmed/33204638 http://dx.doi.org/10.1016/j.ymgmr.2020.100675 |
| _version_ | 1783607366338478080 |
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| author | Cade, W. Todd Bohnert, Kathryn L. Bittel, Adam J. Chacko, Shaji J. Patterson, Bruce W. Pacak, Christina A. Byrne, Barry J. Vernon, Hilary J. Reeds, Dominic N. |
| author_facet | Cade, W. Todd Bohnert, Kathryn L. Bittel, Adam J. Chacko, Shaji J. Patterson, Bruce W. Pacak, Christina A. Byrne, Barry J. Vernon, Hilary J. Reeds, Dominic N. |
| author_sort | Cade, W. Todd |
| collection | PubMed |
| description | Barth syndrome (BTHS) is a rare, X-linked cardiomyopathy that is characterized by abnormalities in glucose and lipid metabolism, with less known regarding amino acid metabolism. This pilot study characterized whole-body arginine kinetics and found lower arginine rate of appearance into plasma (0.69 ± 0.09 vs. 0.88 ± 0.06 μmol/kgFFM/min, p < 0.01) and arginine non-oxidative disposal rate (0.64 ± 0.11 vs. 0.80 ± 0.03 μmol/kgFFM/min, p < 0.02) in adolescents and young adults with BTHS compared to Controls. This study provides a foundation for more in-depth studies on how arginine and potentially other amino acid abnormalities contribute to the pathology and clinical manifestations of BTHS. |
| format | Online Article Text |
| id | pubmed-7649643 |
| institution | National Center for Biotechnology Information |
| language | English |
| publishDate | 2020 |
| publisher | Elsevier |
| record_format | MEDLINE/PubMed |
| spelling | pubmed-76496432020-11-16 Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome Cade, W. Todd Bohnert, Kathryn L. Bittel, Adam J. Chacko, Shaji J. Patterson, Bruce W. Pacak, Christina A. Byrne, Barry J. Vernon, Hilary J. Reeds, Dominic N. Mol Genet Metab Rep Short Communication Barth syndrome (BTHS) is a rare, X-linked cardiomyopathy that is characterized by abnormalities in glucose and lipid metabolism, with less known regarding amino acid metabolism. This pilot study characterized whole-body arginine kinetics and found lower arginine rate of appearance into plasma (0.69 ± 0.09 vs. 0.88 ± 0.06 μmol/kgFFM/min, p < 0.01) and arginine non-oxidative disposal rate (0.64 ± 0.11 vs. 0.80 ± 0.03 μmol/kgFFM/min, p < 0.02) in adolescents and young adults with BTHS compared to Controls. This study provides a foundation for more in-depth studies on how arginine and potentially other amino acid abnormalities contribute to the pathology and clinical manifestations of BTHS. Elsevier 2020-11-04 /pmc/articles/PMC7649643/ /pubmed/33204638 http://dx.doi.org/10.1016/j.ymgmr.2020.100675 Text en © 2020 The Authors. Published by Elsevier Inc. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
| spellingShingle | Short Communication Cade, W. Todd Bohnert, Kathryn L. Bittel, Adam J. Chacko, Shaji J. Patterson, Bruce W. Pacak, Christina A. Byrne, Barry J. Vernon, Hilary J. Reeds, Dominic N. Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome |
| title | Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome |
| title_full | Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome |
| title_fullStr | Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome |
| title_full_unstemmed | Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome |
| title_short | Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome |
| title_sort | arginine kinetics are altered in a pilot sample of adolescents and young adults with barth syndrome |
| topic | Short Communication |
| url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7649643/ https://www.ncbi.nlm.nih.gov/pubmed/33204638 http://dx.doi.org/10.1016/j.ymgmr.2020.100675 |
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