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

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 d...

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Detalles Bibliográficos
Autores principales: Eick, Sigrun, Puklo, Magdalena, Adamowicz, Karina, Kantyka, Tomasz, Hiemstra, Pieter, Stennicke, Henning, Guentsch, Arndt, Schacher, Beate, Eickholz, Peter, Potempa, Jan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4181722/
https://www.ncbi.nlm.nih.gov/pubmed/25260376
http://dx.doi.org/10.1186/s13023-014-0148-y

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