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dc.contributor.authorHryciw, Deanne H
dc.contributor.authorJackson, Courtney A
dc.contributor.authorShrestha, Nirajan
dc.contributor.authorParsons, David
dc.contributor.authorDonnelley, Martin
dc.contributor.authorMcAinch, Andrew J
dc.date.accessioned2021-11-18T01:39:21Z
dc.date.available2021-11-18T01:39:21Z
dc.date.issued2021
dc.identifier.issn1420-682X
dc.identifier.doi10.1007/s00018-021-04014-2
dc.identifier.urihttp://hdl.handle.net/10072/410209
dc.description.abstractEssential fatty acid deficiency has been observed in most patients with Cystic Fibrosis (CF); however, pancreatic supplementation does not restore the deficiency, suggesting a different pathology independent of the pancreas. At this time, the underlying pathological mechanisms are largely unknown. Essential fatty acids are obtained from the diet and processed by organs including the liver and intestine, two organs significantly impacted by mutations in the cystic fibrosis transmembrane conductance regulator gene (Cftr). There are several CF animal models in a variety of species that have been developed to investigate molecular mechanisms associated with the CF phenotype. Specifically, global and systemic mutations in Cftr which mimic genotypic changes identified in CF patients have been generated in mice, rats, sheep, pigs and ferrets. These mutations produce CFTR proteins with a gating defect, trafficking defect, or an absent or inactive CFTR channel. Essential fatty acids are critical to CFTR function, with a bidirectional relationship between CFTR and essential fatty acids proposed. Currently, there are limited analyses on the essential fatty acid status in most of these animal models. Of interest, in the mouse model, essential fatty acid status is dependent on the genotype and resultant phenotype of the mouse. Future investigations should identify an optimal animal model that has most of the phenotypic changes associated with CF including the essential fatty acid deficiencies, which can be used in the development of therapeutics.
dc.description.peerreviewedYes
dc.languageeng
dc.publisherSpringer Science and Business Media LLC
dc.relation.ispartofjournalCellular and Molecular Life Sciences
dc.subject.fieldofresearchBiochemistry and cell biology
dc.subject.fieldofresearchClinical sciences
dc.subject.fieldofresearchMedical biochemistry and metabolomics
dc.subject.fieldofresearchOncology and carcinogenesis
dc.subject.fieldofresearchcode3101
dc.subject.fieldofresearchcode3202
dc.subject.fieldofresearchcode3205
dc.subject.fieldofresearchcode3211
dc.subject.keywordsAnimal models
dc.subject.keywordsArachidonic acid
dc.subject.keywordsCftr
dc.subject.keywordsCystic fibrosis
dc.subject.keywordsDocosahexaenoic acid
dc.titleRole for animal models in understanding essential fatty acid deficiency in cystic fibrosis
dc.typeJournal article
dc.type.descriptionC1 - Articles
dcterms.bibliographicCitationHryciw, DH; Jackson, CA; Shrestha, N; Parsons, D; Donnelley, M; McAinch, AJ, Role for animal models in understanding essential fatty acid deficiency in cystic fibrosis, Cellular and Molecular Life Sciences, 2021
dcterms.dateAccepted2021-10-25
dc.date.updated2021-11-09T22:48:41Z
gro.description.notepublicThis publication has been entered as an advanced online version in Griffith Research Online.
gro.hasfulltextNo Full Text
gro.griffith.authorHryciw, D
gro.griffith.authorHryciw, Deanne


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