Academic Journal
Cystic fibrosis-related metabolic defects: crosstalk between ion channels and organs.
| Τίτλος: | Cystic fibrosis-related metabolic defects: crosstalk between ion channels and organs. |
|---|---|
| Συγγραφείς: | Sims-Lucas S; Department of Pediatrics, and., Goetzman ES; Department of Pediatrics, and., Kleyman TR; Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. |
| Πηγή: | The Journal of clinical investigation [J Clin Invest] 2024 Jul 01; Vol. 134 (13). Date of Electronic Publication: 2024 Jul 01. |
| Τύπος έκδοσης: | Journal Article; Research Support, U.S. Gov't, P.H.S.; Research Support, N.I.H., Extramural |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: American Society for Clinical Investigation Country of Publication: United States NLM ID: 7802877 Publication Model: Electronic Cited Medium: Internet ISSN: 1558-8238 (Electronic) Linking ISSN: 00219738 NLM ISO Abbreviation: J Clin Invest Subsets: MEDLINE |
| Imprint Name(s): | Publication: 1999- : Ann Arbor, MI : American Society for Clinical Investigation Original Publication: New Haven [etc.] American Society for Clinical Investigation. |
| Ιατρικοί όροι (MeSH): | Cystic Fibrosis*/metabolism , Cystic Fibrosis*/genetics , Cystic Fibrosis*/pathology , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics, Animals ; Humans ; Swine ; Disease Models, Animal |
| Περίληψη: | Cystic fibrosis is a debilitating disease characterized by a poor medical prognosis due to devastating lung injury. Recent medical advances targeting the major genetic mutation ΔF508 of the cystic fibrosis transmembrane conductance regulator (CFTR) protein have dramatically increased the lifespan of patients with this mutation. This development has led to major changes in the field and has pushed research beyond the ion transport nature of cystic fibrosis and toward multiorgan physiological reprogramming. In this issue of the JCI, Bae, Kim, and colleagues utilized a large animal pig model prior to the onset of disease. They revealed metabolic reprogramming and organ crosstalk that occurred prior to disease progression. These findings provide paradigm-shifting insight into this complex disease. |
| References: | Lancet. 2016 Nov 19;388(10059):2519-2531. (PMID: 27140670) Cell. 2010 Dec 10;143(6):911-23. (PMID: 21145458) Physiol Rev. 2022 Oct 1;102(4):1625-1667. (PMID: 35378997) Am J Physiol Lung Cell Mol Physiol. 2013 Apr 1;304(7):L469-80. (PMID: 23377346) Eur Respir J. 2004 Jan;23(1):146-58. (PMID: 14738247) Science. 2008 Sep 26;321(5897):1837-41. (PMID: 18818360) Nat Rev Dis Primers. 2015 May 14;1:15010. (PMID: 27189798) Front Endocrinol (Lausanne). 2021 Jul 30;12:702823. (PMID: 34394004) Nutrients. 2021 May 09;13(5):. (PMID: 34065078) Clin Sci (Lond). 2015 Jan;128(2):131-42. (PMID: 25142104) Front Physiol. 2022 Oct 05;13:1019028. (PMID: 36277193) Annu Rev Biochem. 2008;77:701-26. (PMID: 18304008) Lab Anim. 2020 Aug;54(4):330-340. (PMID: 31411127) J Clin Invest. 2024 May 14;134(13):. (PMID: 38743489) J Am Soc Nephrol. 2021 Apr;32(4):897-912. (PMID: 33769951) Adv Exp Med Biol. 2020;1265:71-95. (PMID: 32761571) Lancet Respir Med. 2020 Jan;8(1):65-124. (PMID: 31570318) Kidney Int. 1989 Sep;36(3):351-9. (PMID: 2687561) Children (Basel). 2024 Feb 16;11(2):. (PMID: 38397368) Cochrane Database Syst Rev. 2014 Apr 09;(4):CD005087. (PMID: 24715704) J Biol Chem. 2002 Mar 15;277(11):8928-33. (PMID: 11773060) Lancet. 2021 Jun 5;397(10290):2195-2211. (PMID: 34090606) Life (Basel). 2023 Jul 12;13(7):. (PMID: 37511919) Cold Spring Harb Perspect Med. 2012 Jun;2(6):a009563. (PMID: 22675668) |
| Grant Information: | R01 DK068196 United States DK NIDDK NIH HHS; R01 DK134346 United States DK NIDDK NIH HHS; R01 HL147818 United States HL NHLBI NIH HHS; U54 DK137329 United States DK NIDDK NIH HHS; R01 DK125015 United States DK NIDDK NIH HHS; R25 DK119180 United States DK NIDDK NIH HHS; R25 HL158295 United States HL NHLBI NIH HHS; R01 DK090242 United States DK NIDDK NIH HHS |
| Substance Nomenclature: | 126880-72-6 (Cystic Fibrosis Transmembrane Conductance Regulator) 0 (CFTR protein, human) |
| Entry Date(s): | Date Created: 20240701 Date Completed: 20240701 Latest Revision: 20260803 |
| Update Code: | 20260804 |
| PubMed Central ID: | PMC11213462 |
| DOI: | 10.1172/JCI182329 |
| PMID: | 38949023 |
| Βάση Δεδομένων: | MEDLINE |
| ISSN: | 1558-8238 |
|---|---|
| DOI: | 10.1172/JCI182329 |