Inhibition of CFTR Cl- channel function caused by enzymatic hydrolysis of sphingomyelin.
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ABSTRACT:
SUBMITTER: Ramu Y
PROVIDER: S-EPMC1851081 | biostudies-other | 2007 Apr
REPOSITORIES: biostudies-other
Publications
Inhibition of CFTR Cl- channel function caused by enzymatic hydrolysis of sphingomyelin.
Ramu Yajamana Y Xu Yanping Y Lu Zhe Z
Proceedings of the National Academy of Sciences of the United States of America 20070330 15
Numerous mutations in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR, a Cl(-) channel) disrupt salt and fluid transport and lead to the formation of thick mucus in patients' airways. Obstruction by mucus predisposes CF patients to chronic infections and inflammation, which become gradually harder to control and eventually fatal. Aggressive antibiotic therapy and supportive measures have dramatically lengthened CF patients' lives. Here, we report that sphingomyelinases (SMase) ...[more]
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