Matches in SemOpenAlex for { <https://semopenalex.org/work/W4309171739> ?p ?o ?g. }
- W4309171739 endingPage "14205" @default.
- W4309171739 startingPage "14205" @default.
- W4309171739 abstract "Although cystic fibrosis (CF) is recognized as a monogenic disease, due to variants within the CFTR (Cystic Fibrosis Transmembrane Regulator) gene, an extreme clinical heterogeneity is described among people with CF (pwCF). Apart from the exocrine pancreatic status, most studies agree that there is little association between CFTR variants and disease phenotypes. Environmental factors have been shown to contribute to this heterogeneity, accounting for almost 50% of the variability of the lung function of pwCF. Nevertheless, pwCF with similar CFTR variants and sharing the same environment (such as in siblings) may have highly variable clinical manifestations not explained by CFTR variants, and only partly explained by environmental factors. It is recognized that genetic variants located outside the CFTR locus, named “modifier genes”, influence the clinical expression of the disease. This short review discusses the latest studies that have described modifier factors associated with the various CF phenotypes as well as the response to the recent CFTR modulator therapies." @default.
- W4309171739 created "2022-11-24" @default.
- W4309171739 creator A5001025455 @default.
- W4309171739 creator A5007768330 @default.
- W4309171739 creator A5050814766 @default.
- W4309171739 creator A5052110640 @default.
- W4309171739 date "2022-11-17" @default.
- W4309171739 modified "2023-10-16" @default.
- W4309171739 title "Modifier Factors of Cystic Fibrosis Phenotypes: A Focus on Modifier Genes" @default.
- W4309171739 cites W1589654967 @default.
- W4309171739 cites W1965564449 @default.
- W4309171739 cites W1971977144 @default.
- W4309171739 cites W1973719228 @default.
- W4309171739 cites W1984494027 @default.
- W4309171739 cites W1986398972 @default.
- W4309171739 cites W1992500371 @default.
- W4309171739 cites W1997151909 @default.
- W4309171739 cites W2001314764 @default.
- W4309171739 cites W2007238704 @default.
- W4309171739 cites W2014679781 @default.
- W4309171739 cites W2018617522 @default.
- W4309171739 cites W2021145168 @default.
- W4309171739 cites W2024491038 @default.
- W4309171739 cites W2029523760 @default.
- W4309171739 cites W2031164016 @default.
- W4309171739 cites W2032815975 @default.
- W4309171739 cites W2035131077 @default.
- W4309171739 cites W2036216154 @default.
- W4309171739 cites W2036275580 @default.
- W4309171739 cites W2039599894 @default.
- W4309171739 cites W2041604388 @default.
- W4309171739 cites W2049914201 @default.
- W4309171739 cites W2053102698 @default.
- W4309171739 cites W2062552675 @default.
- W4309171739 cites W2065541938 @default.
- W4309171739 cites W2069947265 @default.
- W4309171739 cites W2072036660 @default.
- W4309171739 cites W2078078890 @default.
- W4309171739 cites W2079477694 @default.
- W4309171739 cites W2086293424 @default.
- W4309171739 cites W2092786611 @default.
- W4309171739 cites W2099222122 @default.
- W4309171739 cites W2104549677 @default.
- W4309171739 cites W2104953797 @default.
- W4309171739 cites W2108658580 @default.
- W4309171739 cites W2109939030 @default.
- W4309171739 cites W2120478186 @default.
- W4309171739 cites W2127267998 @default.
- W4309171739 cites W2129269317 @default.
- W4309171739 cites W2131328125 @default.
- W4309171739 cites W2132859736 @default.
- W4309171739 cites W2140868856 @default.
- W4309171739 cites W2143939104 @default.
- W4309171739 cites W2145394445 @default.
- W4309171739 cites W2149161940 @default.
- W4309171739 cites W2162070501 @default.
- W4309171739 cites W2166496433 @default.
- W4309171739 cites W2168909179 @default.
- W4309171739 cites W2177382009 @default.
- W4309171739 cites W2196566406 @default.
- W4309171739 cites W2206361304 @default.
- W4309171739 cites W2262187954 @default.
- W4309171739 cites W2301131921 @default.
- W4309171739 cites W2302479677 @default.
- W4309171739 cites W2327675250 @default.
- W4309171739 cites W2411292617 @default.
- W4309171739 cites W2413729560 @default.
- W4309171739 cites W2419518172 @default.
- W4309171739 cites W2514448765 @default.
- W4309171739 cites W2564673452 @default.
- W4309171739 cites W2593938141 @default.
- W4309171739 cites W2601786626 @default.
- W4309171739 cites W2607214168 @default.
- W4309171739 cites W2616412773 @default.
- W4309171739 cites W2623866723 @default.
- W4309171739 cites W2738172705 @default.
- W4309171739 cites W2741840231 @default.
- W4309171739 cites W2751826154 @default.
- W4309171739 cites W2765825922 @default.
- W4309171739 cites W2767840926 @default.
- W4309171739 cites W2789525784 @default.
- W4309171739 cites W2803311895 @default.
- W4309171739 cites W2883073823 @default.
- W4309171739 cites W2885178524 @default.
- W4309171739 cites W2914406417 @default.
- W4309171739 cites W2916086954 @default.
- W4309171739 cites W2931836034 @default.
- W4309171739 cites W2977857995 @default.
- W4309171739 cites W2979759357 @default.
- W4309171739 cites W2984381860 @default.
- W4309171739 cites W2985405196 @default.
- W4309171739 cites W2989462885 @default.
- W4309171739 cites W3000213223 @default.
- W4309171739 cites W3009254845 @default.
- W4309171739 cites W3012067044 @default.
- W4309171739 cites W3014434627 @default.
- W4309171739 cites W3035922182 @default.
- W4309171739 cites W3124312347 @default.