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FLASH GENE
Symbol SPPL2A contributors: npt/mct - updated : 17-09-2014
HGNC name signal peptide peptidase like 2A
HGNC id 30227
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestivemouthtoothenamel   Homo sapiensFetal
Endocrineadrenal gland   highly
 parathyroid   highly
Hearing/Equilibriumear   highly
Visualeyeuveachoroid  
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Epithelialbarrier/liningretinal pigment epithelium (RPE)  
cells
SystemCellPubmedSpeciesStageRna symbol
Blood/Hematopoieticleukocyte
Digestiveameloblast Homo sapiensFetal
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
HOMOLOGY
Homologene
FAMILY
  • presenilin-like protein 2 family (I-Clip family)
  • peptidase A22B family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,organelle,endosome
    intracellular,cytoplasm,organelle,lysosome
    basic FUNCTION
  • may be involved in proteolysis and peptidolysis
  • with SPPL2B mediate the intramembrane cleavage, whereas neither SPP nor SPPL3 is capable of processing the ITM2B N-terminal fragment
  • important role for the final step in the CD74-MHC II pathway and a new target for protease inhibitor treatment of B cell diseases
  • enzyme controlling turnover and functions of the invariant chain (CD74) of the MHCII complex, with critical importance of this process for B cell development
  • presenilin-homologue residing in lysosomes/late endosomes, cleaving type II-oriented transmembrane proteins
  • is critical for formation of dental enamel, and intramembrane proteolysis by SPPL2A is essential for maintaining cellular homeostasis of ameloblasts
  • distinct functions of SPPL2A and SPPL2B and, high abundance of SPPL2B in brain suggests a physiological role of this protease in the central nervous system
  • SPPL2A and SPPL2B have been implicated in regulated intramembrane proteolysis (RIP) of type II transmembrane proteins
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacting with ADAM10
  • SPPL2A and SPPL2B mediate the intramembrane cleavage, whereas neither SPP nor SPPL3 is capable of processing the ITM2B N-terminal fragment (pMID: 17965014)
  • aspartyl intramembrane protease, implicated in the proteolysis of TNF, FASLG and ITM2B
  • regulation of CD74-NTF levels by SPPL2A is indispensable for B cell development and function by maintaining trafficking and integrity of MHCII-containing endosomes, highlighting SPPL2a as a promising pharmacological target for depleting and/or modulating B cells
  • SPPL2A, and mainly SPPL2B are responsible for the intramembrane proteolysis of TFRC-N-terminal fragment
  • TMEM106B undergoes regulated intramembrane proteolysis through SPPL2A
  • SPPL2A-mediated processing of CD74 N-terminal fragment (NTF) is indispensable to maintain appropriate levels of tonic BCR signaling to promote B cell maturation
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    ANIMAL & CELL MODELS