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John L. Dart Library
9 a.m. - 5 p.m.
Phone: (843) 722-7550
West Ashley Library
9 a.m. - 5 p.m.
Phone: (843) 766-6635
Folly Beach Library
9 a.m. - 2 p.m.
*open the 2nd and 4th Saturday
*open the 2nd and 4th Saturday
Phone: (843) 588-2001
Edgar Allan Poe/Sullivan's Island Library
Closed for renovations
Phone: (843) 883-3914
Wando Mount Pleasant Library
9 a.m. - 5 p.m.
Phone: (843) 805-6888
Village Library
9 a.m. - 1 p.m.
Phone: (843) 884-9741
St. Paul's/Hollywood Library
9 a.m. - 5 p.m.
Phone: (843) 889-3300
Otranto Road Library
9 a.m. - 5 p.m.
Phone: (843) 572-4094
Mt. Pleasant Library
9 a.m. – 5 p.m.
Phone: (843) 849-6161
McClellanville Library
9 a.m. – 1 p.m.
Phone: (843) 887-3699
Keith Summey North Charleston Library
9 a.m. - 5 p.m.
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John's Island Library
9 a.m. - 5 p.m.
Phone: (843) 559-1945
Hurd/St. Andrews Library
9 a.m. - 5 p.m.
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Miss Jane's Building (Edisto Library Temporary Location)
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Dorchester Road Library
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Phone: (843) 552-6466
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9 a.m. - 5 p.m.
Phone: (843) 795-6679
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9 a.m. - 5 p.m.
Phone: (843) 805-6930
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Phone: (843) 805-6892
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Closed
Phone: (843) 805-6909
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Sheep models of F508del and G542X cystic fibrosis mutations show cellular responses to human therapeutics.
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- Author(s): Viotti Perisse, Iuri; Fan, Zhiqiang; Van Wettere, Arnaud; Liu, Ying; Leir, Shih‐Hsing; Keim, Jacob; Regouski, Misha; Wilson, Michael D.; Cholewa, Kelly M.; Mansbach, Sara N.; Kelley, Thomas J.; Wang, Zhongde; Harris, Ann; White, Kenneth L.; Polejaeva, Irina A.
- Source:
FASEB Bioadvances; Oct2021, Vol. 3 Issue 10, p841-854, 14p - Source:
- Additional Information
- Abstract: Cystic Fibrosis (CF) is a genetic disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. The F508del and G542X are the most common mutations found in US patients, accounting for 86.4% and 4.6% of all mutations, respectively. The F508del causes deletion of the phenylalanine residue at position 508 and is associated with impaired CFTR protein folding. The G542X is a nonsense mutation that introduces a stop codon into the mRNA, thus preventing normal CFTR protein synthesis. Here, we describe the generation of CFTRF508del/F508del and CFTRG542X/G542X lambs using CRISPR/Cas9 and somatic cell nuclear transfer (SCNT). First, we introduced either F508del or G542X mutations into sheep fetal fibroblasts that were subsequently used as nuclear donors for SCNT. The newborn CF lambs develop pathology similar to CFTR−/− sheep and CF patients. Moreover, tracheal epithelial cells from the CFTRF508del/F508del lambs responded to a human CFTR (hCFTR) potentiator and correctors, and those from CFTRG542X/G542X lambs showed modest restoration of CFTR function following inhibition of nonsense‐mediated decay (NMD) and aminoglycoside antibiotic treatments. Thus, the phenotype and electrophysiology of these novel models represent an important advance for testing new CF therapeutics and gene therapy to improve the health of patients with this life‐limiting disorder. [ABSTRACT FROM AUTHOR]
- Abstract: Copyright of FASEB Bioadvances is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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