Unbalanced long-chain fatty acid beta-oxidation in newborns with cystic fibrosis and congenital hypothyroidism

Decreased total long-chain acylcarnitine concentrations in newborns with cystic fibrosis and congenital hypothyroidism suggest diminished beta-oxidation. However, the unexpected relative increase in C18:2 indicates selective preservation of linoleic acid beta-oxidation in both conditions. This is especially surprising in cystic fibrosis where linoleic acid levels become diminished and suggests that linoleic acid beta-oxidation contributes to the deficiency of linoleic acid in cystic fibrosis.

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Publication Details

Journal
Molecular Genetics and Metabolism Reports
Published
2024-12-26
DOI
https://doi.org/10.1016/j.ymgmr.2024.101182
Citations
1
Primary Topic
Metabolism and Genetic Disorders
Type
article
Field-Weighted Citation Impact
0.38

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article

Unbalanced long-chain fatty acid beta-oxidation in newborns with cystic fibrosis and congenital hypothyroidism

Catherina T. Pinnaro, Andrew W. Norris, Kelli K. Ryckman, Aliye Uç
1 citations
Molecular Genetics and Metabolism Reports
Metabolism and Genetic Disorders
0.38
article

Unbalanced long-chain fatty acid beta-oxidation in newborns with cystic fibrosis and congenital hypothyroidism

Catherina T. Pinnaro, Andrew W. Norris, Kelli K. Ryckman, Aliye Uç
article en
1 citations

Abstract

Decreased total long-chain acylcarnitine concentrations in newborns with cystic fibrosis and congenital hypothyroidism suggest diminished beta-oxidation. However, the unexpected relative increase in C18:2 indicates selective preservation of linoleic acid beta-oxidation in both conditions. This is especially surprising in cystic fibrosis where linoleic acid levels become diminished and suggests that linoleic acid beta-oxidation contributes to the deficiency of linoleic acid in cystic fibrosis.

Molecular Genetics and Metabolism ReportsVol. 42
University of Iowa (US), Fraternal Order of Eagles (US), Indiana University Bloomington (US)
U.S. Department of Health and Human Services, Michigan Department of Health and Human Services
Zero hunger
Openalex Percentile: Top 32%
Metabolism and Genetic Disorders
0.38
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Unbalanced long-chain fatty acid beta-oxidation in newborns with cystic fibrosis and congenital hypothyroidism — Catherina T. Pinnaro, Andrew W. Norris, et al. · Molecular Genetics and Metabolism Reports (2024) | TGRS Research Map | TGRS