The Fatty Acid Imbalance of Cystic Fibrosis Exists at Birth Independent of Feeding, in Pig and Ferret Models

Aliye Uc; Birgitta Strandvik; Jianrong Yao; Xiaoming Liu; Yaling Yi; Xingshen Sun; Ruth Welti; John F Engelhardt; Andrew W Norris

doi:10.1042/CS20220450

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The Fatty Acid Imbalance of Cystic Fibrosis Exists at Birth Independent of Feeding, in Pig and Ferret Models

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The Fatty Acid Imbalance of Cystic Fibrosis Exists at Birth Independent of Feeding, in Pig and Ferret Models

Aliye Uc, Birgitta Strandvik, Jianrong Yao, Xiaoming Liu, Yaling Yi, Xingshen Sun, Ruth Welti, John F Engelhardt and Andrew W Norris

Clinical science (1979), Vol.136(24), pp.1773-1791

11/23/2022

DOI: 10.1042/CS20220450

PMCID: PMC9747517

PMID: 36416119

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Abstract

Persons with cystic fibrosis (CF) exhibit a unique alteration of fatty acid composition, marked especially among polyunsaturates by relative deficiency of linoleic acid and excess of Mead acid. Relative deficiency of docosahexaenoic acid is variably found. However, the initial development of these abnormalities is not understood. We examined fatty acid composition in young CF ferrets and pigs, finding abnormalities from the day of birth onward including relative deficiency of linoleic acid in both species. Fatty acid composition abnormalities were present in both liver and serum phospholipids of newborn CF piglets even prior to feeding, including reduced linoleic acid and increased Mead acid. Serum fatty acid composition evolved over the first weeks of life in both non-CF and CF ferrets, though differences between CF and non-CF persisted. Although red blood cell phospholipid fatty acid composition was normal in newborn animals, it became perturbed in juvenile CF ferrets including relative deficiencies of linoleic and docosahexaenoic acids and excess of Mead acid. In summary, fatty acid composition abnormalities in CF pigs and ferrets exist from a young age including at birth independent of feeding and overlap extensively with the abnormalities found in humans with CF. That the abnormalities exist prior to feeding implies that dietary measures alone will not address the mechanisms of imbalance.

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Title: Subtitle: The Fatty Acid Imbalance of Cystic Fibrosis Exists at Birth Independent of Feeding, in Pig and Ferret Models
Creators: Aliye Uc - United States
Birgitta Strandvik - Karolinska Institutet
Jianrong Yao - Roy J. and Lucille A. Carver College of Medicine
Xiaoming Liu - Roy J. and Lucille A. Carver College of Medicine
Yaling Yi - Roy J. and Lucille A. Carver College of Medicine
Xingshen Sun - Roy J. and Lucille A. Carver College of Medicine
Ruth Welti - Kansas State University
John F Engelhardt - Roy J. and Lucille A. Carver College of Medicine
Andrew W Norris - Roy J. and Lucille A. Carver College of Medicine
Resource Type: Journal article
Publication Details: Clinical science (1979), Vol.136(24), pp.1773-1791
DOI: 10.1042/CS20220450
PMID: 36416119
PMCID: PMC9747517
NLM abbreviation: Clin Sci (Lond)
ISSN: 0143-5221
eISSN: 1470-8736
Language: English
Date published: 11/23/2022
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Endocrinology and Diabetes; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Radiation Oncology; Gastroenterology, Hepatology, Pancreatology, and Nutrition; Biochemistry and Molecular Biology; Internal Medicine
Record Identifier: 9984320159502771

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