TY  - JOUR
AU  - Bronckers, Antonius L J J
AU  - Gueneli, Nur
AU  - Lüllmann-Rauch, Renate
AU  - Schneppenheim, Janna
AU  - Moraru, Andreea P
AU  - Himmerkus, Nina
AU  - Bervoets, Theodore J
AU  - Fluhrer, Regina
AU  - Everts, Vincent
AU  - Saftig, Paul
AU  - Schröder, Bernd
TI  - The intramembrane protease SPPL2A is critical for tooth enamel formation.
JO  - Journal of bone and mineral research
VL  - 28
IS  - 7
SN  - 0884-0431
CY  - Hoboken, NJ [u.a.]
PB  - Wiley
M1  - DZNE-2020-03266
SP  - 1622-1630
PY  - 2013
AB  - 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
KW  - Ameloblasts: enzymology
KW  - Animals
KW  - Antigens, Differentiation, B-Lymphocyte: genetics
KW  - Antigens, Differentiation, B-Lymphocyte: metabolism
KW  - Aspartic Acid Endopeptidases: genetics
KW  - Aspartic Acid Endopeptidases: metabolism
KW  - Dental Enamel: enzymology
KW  - Dental Enamel: growth & development
KW  - Histocompatibility Antigens Class II: genetics
KW  - Histocompatibility Antigens Class II: metabolism
KW  - Incisor: enzymology
KW  - Incisor: growth & development
KW  - Membrane Proteins: genetics
KW  - Membrane Proteins: metabolism
KW  - Mice
KW  - Mice, Knockout
KW  - Proteolysis
KW  - Antigens, Differentiation, B-Lymphocyte (NLM Chemicals)
KW  - Histocompatibility Antigens Class II (NLM Chemicals)
KW  - Membrane Proteins (NLM Chemicals)
KW  - invariant chain (NLM Chemicals)
KW  - Aspartic Acid Endopeptidases (NLM Chemicals)
KW  - SPPL2a protein, mouse (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:23426979
DO  - DOI:10.1002/jbmr.1895
UR  - https://pub.dzne.de/record/136944
ER  -