We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Cathepsin C, ein Aktivator von Serinproteasen - Einfluss auf die Pathogenese von Papillon-Lefèvre-Syndrom und Parodontitis: Diskussionsbeitrag des Masterkurses „Parodontologie und Implantattherapie" der DG PARO und DIU.
- Authors
Gaß, Johanna; Götze, Felix; Grou, Tudor; Hackbarth, Christoph; Rotter, Michael; Schneider, Lena; Schneider, Sophia; Moghaddam, Schiwa Seyedi; Herrmann, Jens M.
- Abstract
The cysteine protease cathepsin C plays an important role in the host response through its activation of serine proteases in intrinsic immune and inflammatory cells, as well as the degradation of proteins. Numerous mutations of the cathepsin C gene are associated with Papillon-Lefèvre syndrome (PLS), an autosomal recessive hereditary palmoplantar keratosis. The lack of activation of several serine proteases, due to the deficiency of cathepsin C caused by the mutation, lead to functional defects of various immune cells. Of these, research has focused on polymorphonuclear neutrophils (PMN), which exhibit an increased recruitment in inflamed tissues. Furthermore, the decreased tissue transit time of PMN and concomitantly decreased antimicrobial functions explain the severity of periodontitis in patients suffering from PLS. Through this central linkage in immune reactions, cathepsin C has become the focus of research on therapeutic interventions in inflammatory diseases. As a result, primarily synthetic inhibitors of cathepsin C have been developed. However, Papillon-Lefèvre syndrome cannot be treated with these inhibitors, as they weaken cathepsin C activity. Further research is needed, preferably on a genetic basis, to find ways of compensating for the typical deficiency of cathepsin C in Papillon-Lefèvre syndrome.
- Subjects
SERINE proteinases; PROTEOLYSIS; NEUTROPHILS; KERATOSIS; CYSTEINE; PERIODONTITIS
- Publication
Parodontologie: die Zeitschrift für die Praxis (Berlin, Germany), 2022, Vol 33, Issue 4, p461
- ISSN
0937-1532
- Publication type
Article