2017
DOI: 10.1007/s00109-017-1577-6
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Cystic fibrosis: the conductance regulator, ceramides, and possible treatments

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Cited by 4 publications
(4 citation statements)
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“…Furthermore, rosiglitazone had positive effects in CFTR −/− mice, including increased bicarbonate secretion and reduction of mucus in the intestine ( 147 ). Early promising results in mice with rosiglitazone prompted speculation that it could be used in pwCF, but due to severe side effects with long-term use, rosiglitazone has been removed from the market in several countries ( 148 150 ). However, PPAR-γ remains an interesting potential target for improving CF outcomes.…”
Section: Drivers Of Microbiota Alterationsmentioning
confidence: 99%
“…Furthermore, rosiglitazone had positive effects in CFTR −/− mice, including increased bicarbonate secretion and reduction of mucus in the intestine ( 147 ). Early promising results in mice with rosiglitazone prompted speculation that it could be used in pwCF, but due to severe side effects with long-term use, rosiglitazone has been removed from the market in several countries ( 148 150 ). However, PPAR-γ remains an interesting potential target for improving CF outcomes.…”
Section: Drivers Of Microbiota Alterationsmentioning
confidence: 99%
“…Opposing data exist regarding the role of Cer and C1P in fibrosis. Although Cer has been shown to attenuate lipopolysaccharide response in macrophages (321), it has also been linked to chronic inflammation in adipose tissue (322), whereas inhibition of acid SMase reduces pulmonary fibrosis in cystic fibrosis (323,324). In turn, C1P displays anti-inflammatory functions in chronic obstructive pulmonary disease, where it attenuates inflammation and fibrosis (325).…”
Section: Sphingolipids: Unlocking the Pathways To Fibrotic Disorders mentioning
confidence: 99%
“…One study by Utermöhlen et al demonstrated that acid sphingomyelinase-deficient mice are more resistant to lethal infections with M. avium than wild-type mice ( 41 ). Ceramides in CF are generally important for the regulation of cytokines and inflammation, but as with itaconate, some conflicting evidence exists ( 42 , 43 ). Some studies have found that ceramides accumulate in the CF airways, which in turn causes inflammation and an increased susceptibility to bacterial infections ( 42 ).…”
Section: Discussionmentioning
confidence: 99%
“…Ceramides in CF are generally important for the regulation of cytokines and inflammation, but as with itaconate, some conflicting evidence exists ( 42 , 43 ). Some studies have found that ceramides accumulate in the CF airways, which in turn causes inflammation and an increased susceptibility to bacterial infections ( 42 ). Conversely, other studies have found that the increased presence of ceramides in CF patients helps to reduce overall inflammation and cytokine production in the lungs ( 44 ).…”
Section: Discussionmentioning
confidence: 99%