Lack of cathelicidin processing in Papillon-Lefèvre syndrome patients reveals essential role of LL-37 in periodontal homeostasis

@inproceedings{Eick2014LackOC,
  title={Lack of cathelicidin processing in Papillon-Lef{\`e}vre syndrome patients reveals essential role of LL-37 in periodontal homeostasis},
  author={Sigrun Eick and Magdalena Puklo and Karina Adamowicz and Tomasz Kantyka and Pieter S Hiemstra and Henning Ralf Stennicke and Arndt Guentsch and Beate Schacher and Peter Eickholz and Jan Potempa},
  booktitle={Orphanet journal of rare diseases},
  year={2014}
}
BACKGROUND Loss-of-function point mutations in the cathepsin C gene are the underlying genetic event in patients with Papillon-Lefèvre syndrome (PLS). PLS neutrophils lack serine protease activity essential for cathelicidin LL-37 generation from hCAP18 precursor. AIM We hypothesized that a local deficiency of LL-37 in the infected periodontium is mainly responsible for one of the clinical hallmark of PLS: severe periodontitis already in early childhood. METHODS To confirm this effect, we… CONTINUE READING
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