Periodontal Disease in Patients From the Original Kostmann Family With Severe Congenital Neutropenia

Carlsson, Göran; Wahlin, Ylva-Britt; Johansson, Anders; Olsson, A T; Eriksson, Torbjörn; Claesson, Rolf; Hänström, Lennart; Henter, Jan‐Inge

doi:10.1902/jop.2006.050191

Cited by 102 publications

(79 citation statements)

References 30 publications

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“…Decreased CAMP/LL-37 production accompanies increased invasion and colonization by pathogens in epithelial cells, which characterizes diseases such as morbus Kostmann (15) and atopic dermatitis (16).…”

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confidence: 99%

Augmentation of Epithelial Resistance to Invading Bacteria by Using mRNA Transfections

et al. 2013

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bTo protect against invading bacteria, oral epithelial cells appear to use two effector antimicrobial peptides (AMPs): calprotectin (S100A8-S100A9 heterodimer [S100A8/A9]) in the cytosol and cathelicidin antimicrobial protein (CAMP) in endosomes. We sought to learn whether innate immunity might be augmented benignly to increase resistance against invasive bacteria. Epithelial cells were transiently transfected with mRNA constructs containing either the CAMP, S100A8, and S100A9 open reading frames, A8-IRES-A9 (fusion sequence), or A8-nIRES-A9 (fusion with native internal ribosome entry site [IRES] sequence). CAMP, S100A8, and S100A9 protein levels generally peaked between 16 and 44 h after mRNA transfection, depending on the construct; CAMP was processed to LL-37 over time. Following transfection with the respective mRNAs, CAMP and S100A8/A9 each independently increased resistance of epithelial cells to invasion by Listeria and Salmonella for up to 48 h; tandem S100A8/A9 constructs were also effective. Cotransfection to express S100A8/A9 and CAMP together augmented resistance, but synergy was not seen. Independent of the new proteins produced, transfection reduced cell viability after 48 h by 20%, with only 2% attributable to apoptosis. Taken together, these results suggest that epithelial cell resistance to invasive pathogens can be augmented by transient transfection of antimicrobial mRNAs into epithelial cells.

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Augmentation of Epithelial Resistance to Invading Bacteria by Using mRNA Transfections

et al. 2013

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“…LL-37 has broad-spectrum activity against Gram-positive and Gram-negative microorganisms, including those associated with periodontal disease (27). A rare disorder, morbus Kostmann, is associated with the complete absence of LL-37 and is characterized by, among other symptoms, chronic periodontitis and overgrowth with A. actinomycetemcomitans (8,41). Individuals with another genetic disorder, Papillon-Lefèvre syndrome, demonstrate a deficiency in LL-37 and exhibit severe periodontitis.…”

Section: Discussionmentioning

confidence: 99%

“…LL-37 gene expression can be induced by live bacteria (36) or by bacterial products such as lipopolysaccharide (LPS) (30,39), and the active LL-37 peptide is processed from the inactive human CAP-18 (hCAP-18) precursor by proteolysis. Lack of LL-37 is associated with two human disorders, morbus Kostmann and Papillon-Lefèvre syndrome, in which there is severe periodontal disease associated with colonization by the periodontal pathogen Aggregatibacter actinomycetemcomitans (8,11,41). LL-37 gene expression can be induced by live bacteria (36) or by bacterial products such as LPS (30,39), making the peptide part of the innate immune defense of the gingival epithelium.…”

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confidence: 99%

Vitamin D-Mediated Induction of Innate Immunity in Gingival Epithelial Cells

et al. 2011

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“…Patients may sufer from recurrent gingivitis and even severe periodontitis [94]. To have a family history of consanguineous parenthood may be a predisposing factor for infantile genetic agranulocytosis [95].…”

Section: Genetic Disorders With Periodontal Manifestations and Consanmentioning

confidence: 99%

Oral and Periodontal Diseases in Consanguineous Marriages

Çalışır¹

2017

Insights Into Various Aspects of Oral Health

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Periodontitis is deined as an inlammatory disease of supporting tissues of teeth characterized by progressive destruction of the periodontal ligament and alveolar bone. Periodontal manifestations of these genetic disorders or syndromes, such as familial and cyclic neutropenias, granulomatous disease, agranulocytosis, Langerhans' cell disease, glycogen storage disease, hypophosphatasia, leucocyte adhesion deiciency, and Papillon-Lefèvre, Chédiak-Higashi, Cohen, Ehlers-Danlos, Marfan, Down, Haim-Munk, and Kindlers syndromes, imitate some types of periodontal diseases. Most of these syndromes have autosomal-recessive characterization and can be seen commonly in consanguineous marriages. Therefore, consanguineous marriages have generally been accepted as having important detrimental efects on ofspring. There is a lot of genetic research about consanguineous marriage and its detrimental efects on ofspring. Although consanguineous marriages are common in the world, the relationship with oral and periodontal diseases has not been thoroughly investigated. We do not have enough of an understanding of the efects of consanguineous marriage on oral and periodontal diseases. In this chapter, previous studies in the literature related to this subject will be investigated and evaluated, and then this research will be related to oral and periodontal diseases. Therefore, this chapter will guide further research. The aim of this chapter is to show the relation between consanguineous marriages and oral-periodontal diseases.

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Periodontal Disease in Patients From the Original Kostmann Family With Severe Congenital Neutropenia

Cited by 102 publications

References 30 publications

Augmentation of Epithelial Resistance to Invading Bacteria by Using mRNA Transfections

Augmentation of Epithelial Resistance to Invading Bacteria by Using mRNA Transfections

Vitamin D-Mediated Induction of Innate Immunity in Gingival Epithelial Cells

Oral and Periodontal Diseases in Consanguineous Marriages

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