Role for animal models in understanding essential fatty acid deficiency in cystic fibrosis

Hryciw, Deanne H.; Jackson, Courtney A.; Shrestha, Nirajan; Parsons, David; Donnelley, Martin; McAinch, Andrew J.

doi:10.1007/s00018-021-04014-2

Cited by 3 publications

(6 citation statements)

References 65 publications

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“…Despite this, the fatty acid analyses may have been influenced by food consumption prior to blood sample collection. Compared to the CF mice models, with differences in EFA status based on the genetic background [ 10 ], the increased AA:DHA ratio in serum of the CF Phe508del rat suggests that this model is closer to patients with CF, despite LA concentrations in the serum being unchanged.…”

Section: Discussionmentioning

confidence: 99%

“…PUFAs can be classified into two main types—omega-3 (n-3) and omega-6 (n-6) FA, which contain the EFAs α-linolenic acid (ALA) and linoleic acid (LA), respectively [ 10 ]. ALA and LA cannot be synthesized in humans and must be obtained via the diet.…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…Limited research has investigated the molecular mechanism that gives rise to EFA deficiency [ 10 ] in animal models of CF [ 15 ]. In our recent review, we summarized the EFA status in animal models of CF [ 10 ]. Within these models, where the EFA status has been characterized, the EFA concentrations do not mimic what is observed in patients with CF [ 10 ].…”

Section: Introductionmentioning

confidence: 99%

“…In our recent review, we summarized the EFA status in animal models of CF [ 10 ]. Within these models, where the EFA status has been characterized, the EFA concentrations do not mimic what is observed in patients with CF [ 10 ]. A recently developed animal model, the CF rat, has several characteristics similar to patients with CF.…”

Section: Introductionmentioning

confidence: 99%

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Changes in Essential Fatty Acids and Ileal Genes Associated with Metabolizing Enzymes and Fatty Acid Transporters in Rodent Models of Cystic Fibrosis

Shrestha

Rout-Pitt

McCarron

et al. 2023

IJMS

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Cystic fibrosis (CF), the result of mutations in the CF transmembrane conductance regulator (CFTR), causes essential fatty acid deficiency. The aim of this study was to characterize fatty acid handling in two rodent models of CF; one strain which harbors the loss of phenylalanine at position 508 (Phe508del) in CFTR and the other lacks functional CFTR (510X). Fatty acid concentrations were determined using gas chromatography in serum from Phe508del and 510X rats. The relative expression of genes responsible for fatty acid transport and metabolism were quantified using real-time PCR. Ileal tissue morphology was assessed histologically. There was an age-dependent decrease in eicosapentaenoic acid and the linoleic acid:α-linolenic acid ratio, a genotype-dependent decrease in docosapentaenoic acid (n-3) and an increase in the arachidonic acid:docosahexaenoic acid ratio in Phe508del rat serum, which was not observed in 510X rats. In the ileum, Cftr mRNA was increased in Phe508del rats but decreased in 510X rats. Further, Elvol2, Slc27a1, Slc27a2 and Got2 mRNA were increased in Phe508del rats only. As assessed by Sirius Red staining, collagen was increased in Phe508del and 510X ileum. Thus, CF rat models exhibit alterations in the concentration of circulating fatty acids, which may be due to altered transport and metabolism, in addition to fibrosis and microscopic structural changes in the ileum.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%