Defective CFTR increases synthesis and mass of sphingolipids that modulate membrane composition and lipid signaling

Defective CFTR increases synthesis and mass of sphingolipids that modulate membrane composition and lipid signaling

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) that affect protein structure and channel function. CFTR, localized in the apical membrane within cholesterol and sphingomyelin rich regions, is an ABC transporter that functions as a chloride channel. H...

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Bibliographic Details
Main Authors: Hiroko Hamai, Fannie Keyserman, Lynne M. Quittell, Tilla S. Worgall
Format: Article
Language:English
Published: Elsevier 2009-06-01
Series:Journal of Lipid Research
Subjects:
cystic fibrosis transmembrane conductance regulator
sphingomyelin
sphingosine
sphinganine
serine-palmitoyl transferase
long-chain base subunit 1
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520308087

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http://www.sciencedirect.com/science/article/pii/S0022227520308087

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