Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome.
Publication
, Journal Article
Cade, WT; Bohnert, KL; Bittel, AJ; Chacko, SJ; Patterson, BW; Pacak, CA; Byrne, BJ; Vernon, HJ; Reeds, DN
Published in: Mol Genet Metab Rep
December 2020
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.
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Published In
Mol Genet Metab Rep
DOI
ISSN
2214-4269
Publication Date
December 2020
Volume
25
Start / End Page
100675
Location
United States
Related Subject Headings
- 3202 Clinical sciences
- 3105 Genetics
- 0604 Genetics
- 0601 Biochemistry and Cell Biology
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APA
Chicago
ICMJE
MLA
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Cade, W. T., Bohnert, K. L., Bittel, A. J., Chacko, S. J., Patterson, B. W., Pacak, C. A., … Reeds, D. N. (2020). Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome. Mol Genet Metab Rep, 25, 100675. https://doi.org/10.1016/j.ymgmr.2020.100675
Cade, W Todd, Kathryn L. Bohnert, Adam J. Bittel, Shaji J. Chacko, Bruce W. Patterson, Christina A. Pacak, Barry J. Byrne, Hilary J. Vernon, and Dominic N. Reeds. “Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome.” Mol Genet Metab Rep 25 (December 2020): 100675. https://doi.org/10.1016/j.ymgmr.2020.100675.
Cade WT, Bohnert KL, Bittel AJ, Chacko SJ, Patterson BW, Pacak CA, et al. Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome. Mol Genet Metab Rep. 2020 Dec;25:100675.
Cade, W. Todd, et al. “Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome.” Mol Genet Metab Rep, vol. 25, Dec. 2020, p. 100675. Pubmed, doi:10.1016/j.ymgmr.2020.100675.
Cade WT, Bohnert KL, Bittel AJ, Chacko SJ, Patterson BW, Pacak CA, Byrne BJ, Vernon HJ, Reeds DN. Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome. Mol Genet Metab Rep. 2020 Dec;25:100675.
Published In
Mol Genet Metab Rep
DOI
ISSN
2214-4269
Publication Date
December 2020
Volume
25
Start / End Page
100675
Location
United States
Related Subject Headings
- 3202 Clinical sciences
- 3105 Genetics
- 0604 Genetics
- 0601 Biochemistry and Cell Biology