ArticleOrphanet journal of rare diseases2014
Lack of cathelicidin processing in Papillon-Lefèvre syndrome patients reveals essential role of LL-37 in periodontal homeostasis.
Article in Orphanet journal of rare diseases, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 45 citations in OpenAlex.
- Functions of Macrophages in Periodontal Disease Progression and Gingival Tissue Homeostasis.Advances in experimental medicine and biology · 2026Review
- Differences in Inflammatory Genetic Profiles in Periodontitis Associated with Genetic and Immunological Disorders: A Systematic Review.Biomedicines · 2025Review
- Porphyromonas gingivalis outer membrane vesicles divert host innate immunity and promote inflammation via C4' monophosphorylated lipid A.Journal of immunology (Baltimore, Md. : 1950) · 2025Article
- Inborn errors of immunity-related immunological mechanisms and pharmacological therapy alternatives in periodontitis.Clinical and experimental immunology · 2025Review
- Targeting neutrophil serine proteases in bronchiectasis.The European respiratory journal · 2025Review
- The oral microbiome of a family including Papillon-Lefèvre-syndrome patients and clinically healthy members.BMC oral health · 2024Article
- Subsiding of Periodontitis in the Permanent Dentition in Individuals with Papillon-Lefèvre Syndrome through Specific Periodontal Treatment: A Systematic Review.Healthcare (Basel, Switzerland) · 2022Article
- Riemerella anatipestifer T9SS Effector SspA Functions in Bacterial Virulence and Defending Natural Host Immunity.Applied and environmental microbiology · 2022Article
- Cathelicidin LL-37 in Health and Diseases of the Oral Cavity.Biomedicines · 2022Review
- Citrullination Alters the Antiviral and Immunomodulatory Activities of the Human Cathelicidin LL-37 During Rhinovirus Infection.Frontiers in immunology · 2020Article
- GPN does not release lysosomal CaJournal of cell science · 2019Article
- Antimicrobial peptide LL-37 and recombinant human mannose-binding lectin express distinct age- and pathogen-specific antimicrobial activity in human newborn cord bloodF1000Research · 2018Article
- Significance and Diagnostic Role of Antimicrobial Cathelicidins (LL-37) Peptides in Oral Health.Biomolecules · 2017Review
- Gingival crevicular fluid and serum hCAP18/LL-37 levels in generalized aggressive periodontitis.Clinical oral investigations · 2017Article
- Levels of ll-37 antimicrobial peptide in the gingival crevicular fluid of young and middle-aged subjects with or without gingivitis.Journal of Istanbul University Faculty of Dentistry · 2017Article
- Manipulation of Neutrophils byFrontiers in cellular and infection microbiology · 2017Review
- Human neutrophils and oral microbiota: a constant tug-of-war between a harmonious and a discordant coexistence.Immunological reviews · 2016Review
- Neutrophil extracellular traps - the dark side of neutrophils.The Journal of clinical investigation · 2016Review
- Immune and regulatory functions of neutrophils in inflammatory bone loss.Seminars in immunology · 2016Review
- Neutrophil Functions in Periodontal Homeostasis.Journal of immunology research · 2016Review
Corrections and comments
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Authors and funding
10 authors at 6 institutions in 6 countries.
Funding
Abstract
backgroundLoss-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.
aimWe 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.
methodsTo confirm this effect, we compared the level of neutrophil-derived enzymes and antimicrobial peptides in gingival crevicular fluid (GCF) and saliva from PLS, aggressive and chronic periodontitis patients.
resultsAlthough neutrophil numbers in GCF were present at the same level in all periodontitis groups, LL-37 was totally absent in GCF from PLS patients despite the large amounts of its precursor, hCAP18. The absence of LL-37 in PLS patients coincided with the deficiency of both cathepsin C and protease 3 activities. The presence of other neutrophilic anti-microbial peptides in GCF from PLS patients, such as alpha-defensins, were comparable to that found in chronic periodontitis. In PLS microbial analysis revealed a high prevalence of Aggregatibacter actinomycetemcomitans infection. Most strains were susceptible to killing by LL-37.
conclusionsCollectively, these findings imply that the lack of protease 3 activation by dysfunctional cathepsin C in PLS patients leads to the deficit of antimicrobial and immunomodulatory functions of LL-37 in the gingiva, allowing for infection with A. actinomycetemcomitans and the development of severe periodontal disease.
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