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Aberrant Cell Biology in Cystic Fibrosis Cells. Edition No. 1

VDM Publishing House, June 2009, Pages: 132


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This book describes a novel cell biological basis
contributing to aberrant innate immunity and
inflammation in the cystic fibrosis (CF) respiratory
tract. The first aspect covered involves the basis
for previously described hyperacidification of
organelles in CF cells, and how this phenomenon can
be pharmacologically corrected. The second aspect
focuses on the excessive production of a molecule
named Sialyl-Lewis X (Slx) in CF cells. Slx acts as
a receptor for neutrophils which cause excessive
damage in the CF bronchial epithelia. The third
aspect involves the discovery of attenuated
polymeric IgA transcytosis in CF cells, which may
contribute to excessive colonization of P.
aeruginosa. Future directions addressing these
aspects of the cell biology of CF are outlined.



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