%0 Journal Article
%A Bronckers, Antonius L J J
%A Gueneli, Nur
%A Lüllmann-Rauch, Renate
%A Schneppenheim, Janna
%A Moraru, Andreea P
%A Himmerkus, Nina
%A Bervoets, Theodore J
%A Fluhrer, Regina
%A Everts, Vincent
%A Saftig, Paul
%A Schröder, Bernd
%T The intramembrane protease SPPL2A is critical for tooth enamel formation.
%J Journal of bone and mineral research
%V 28
%N 7
%@ 0884-0431
%C Hoboken, NJ [u.a.]
%I Wiley
%M DZNE-2020-03266
%P 1622-1630
%D 2013
%X Intramembrane proteases are critically involved in signal transduction and membrane protein turnover. Signal-peptide-peptidase-like 2a (SPPL2A), a presenilin-homologue residing in lysosomes/late endosomes, cleaves type II-oriented transmembrane proteins. We recently identified SPPL2A as the enzyme controlling turnover and functions of the invariant chain (CD74) of the major histocompatibility complex II (MHCII) and demonstrated critical importance of this process for B cell development. Surprisingly, we found that SPPL2A is critical for formation of dental enamel. In Sppl2a knockout mice, enamel of the erupted incisors was chalky white and rapidly eroded after eruption. SPPL2A was found to be expressed in enamel epithelium during secretory and maturation stage amelogenesis. Mineral content of enamel in Sppl2a⁻/⁻ incisors was inhomogeneous and reduced by ∼20
%K Ameloblasts: enzymology
%K Animals
%K Antigens, Differentiation, B-Lymphocyte: genetics
%K Antigens, Differentiation, B-Lymphocyte: metabolism
%K Aspartic Acid Endopeptidases: genetics
%K Aspartic Acid Endopeptidases: metabolism
%K Dental Enamel: enzymology
%K Dental Enamel: growth & development
%K Histocompatibility Antigens Class II: genetics
%K Histocompatibility Antigens Class II: metabolism
%K Incisor: enzymology
%K Incisor: growth & development
%K Membrane Proteins: genetics
%K Membrane Proteins: metabolism
%K Mice
%K Mice, Knockout
%K Proteolysis
%K Antigens, Differentiation, B-Lymphocyte (NLM Chemicals)
%K Histocompatibility Antigens Class II (NLM Chemicals)
%K Membrane Proteins (NLM Chemicals)
%K invariant chain (NLM Chemicals)
%K Aspartic Acid Endopeptidases (NLM Chemicals)
%K SPPL2a protein, mouse (NLM Chemicals)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:23426979
%R 10.1002/jbmr.1895
%U https://pub.dzne.de/record/136944