Lack of cathelicidin processing in Papillon-Lefèvre syndrome patients reveals essential role of LL-37 in periodontal homeostasis
نویسندگان
چکیده
منابع مشابه
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 deficiency of LL-37 in the infected periodontium is mainly responsible for one of the clinical hal...
متن کاملPreventive periodontal regimen in Papillon-Lefèvre syndrome.
PURPOSE The purpose of this study was to evaluate the effect of a comprehensive preventive program, based on mechanical plaque control and local and systemic antibacterial measures, on periodontal health and preservation of permanent teeth in patients with Papillon-Lefèvre syndrome (PLS). METHODS Thirty-five consecutive PLS patients (median age=7 years; range=3-19 years) were treated and foll...
متن کاملPapillon-Lefèvre syndrome.
Papillon- Lefèvre syndrome (PLS) is a rare autosomal recessive disorder of keratinization characterized by palmoplantar hyperkeratosis, periodontopathy and precocious loss of dentition. The exact pathomechanism of these clinical events mainly remains speculative. This paper describes two cases of PLS with classic clinical features and briefly review the relevant literature.
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INTRODUCTION Literature regarding oral conditions in patients with Papillon-Lefèvre syndrome (PLS) often covers the periodontal aspects, but no literature was found describing specific craniofacial findings in this group. The aim of this retrospective study was to investigate the cephalometric findings of patients with PLS. METHODS Lateral cephalograms of 8 patients with PLS were traced, and ...
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Background Rotavirus is the most prevalent cause of severe gastroenteritis, hospitalizations, and deaths among infants and young children, globally. No specific antiviral drug is available against rotavirus infection. Objective The current study aimed to assess the antiviral effect of human cathelicidin antimicrobial peptide LL-37 on rotavirus infection in vitro. Methods This study was con...
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ژورنال
عنوان ژورنال: Orphanet Journal of Rare Diseases
سال: 2014
ISSN: 1750-1172
DOI: 10.1186/s13023-014-0148-y