TY - JOUR AU - Mentrup, Torben AU - Cabrera-Cabrera, Florencia AU - Fluhrer, Regina AU - Schröder, Bernd TI - Physiological functions of SPP/SPPL intramembrane proteases JO - Cellular and molecular life sciences VL - 77 IS - 15 SN - 1420-682X CY - Cham (ZG) PB - Springer International Publishing AG M1 - DZNE-2020-01139 SP - 2959-2979 PY - 2020 AB - Intramembrane proteolysis describes the cleavage of substrate proteins within their hydrophobic transmembrane segments. Several families of intramembrane proteases have been identified including the aspartyl proteases Signal peptide peptidase (SPP) and its homologues, the SPP-like (SPPL) proteases SPPL2a, SPPL2b, SPPL2c and SPPL3. As presenilin homologues, they employ a similar catalytic mechanism as the well-studied γ-secretase. However, SPP/SPPL proteases cleave transmembrane proteins with a type II topology. The characterisation of SPP/SPPL-deficient mouse models has highlighted a still growing spectrum of biological functions and also promoted the substrate discovery of these proteases. In this review, we will summarise the current hypotheses how phenotypes of these mouse models are linked to the molecular function of the enzymes. At the cellular level, SPP/SPPL-mediated cleavage events rather provide specific regulatory switches than unspecific bulk proteolysis. By this means, a plethora of different cell biological pathways is influenced including signal transduction, membrane trafficking and protein glycosylation. KW - Amyloid Precursor Protein Secretases: metabolism KW - Animals KW - Aspartic Acid Endopeptidases: chemistry KW - Aspartic Acid Endopeptidases: metabolism KW - Humans KW - Membrane Proteins: metabolism KW - Protein Transport KW - Proteolysis KW - Signal Transduction KW - Substrate Specificity LB - PUB:(DE-HGF)16 C6 - pmid:32052089 C2 - pmc:PMC7366577 DO - DOI:10.1007/s00018-020-03470-6 UR - https://pub.dzne.de/record/151555 ER -