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FLASH GENE
Symbol RCE1 contributors: mct/npt/pgu - updated : 08-07-2009
HGNC name RCE1 homolog, prenyl protein protease (S. cerevisiae)
HGNC id 13721
Location 11q13.1      Physical location : 66.610.882 - 66.614.003
Genatlas name RAS-converting enzyme 1
Synonym name
  • prenyl protein protease RCE1B
  • farnesylated protein-converting enzyme 2
  • RCE1 (S. Cerevisiae) homolog, prenyl protein protease
  • Synonym symbol(s) FACE2, HRCE1, RCE1A, RCE1B
    EC.number 3.4.22.-
    DNA
    TYPE functioning gene
    STRUCTURE 3.12 kb     8 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Physical map
    SLC29A2 11q13 solute carrier family 29 (nucleoside transporters), member 2 LOC387784 11 LOC387784 NXF 11q13 HLH-PAS transcription factor NXF MRPL11 11q13.3 mitochondrial ribosomal protein L11 MGC35521 11q13.1 pellino 3 alpha DPP3 11q12-q13.1 dipeptidylpeptidase 3 BBS1 11q13 Bardet-Biedl syndrome 1 LOC254359 11q13.1 hypothetical protein LOC254359 ACTN3 11q13 actinin, alpha 3 CTSF 11q13.1-q13.3 cathepsin F FLJ10786 11q13.1 hypothetical protein FLJ10786 CCS 11q13 copper chaperone for superoxide dismutase RBM14 11q12.1 RNA binding motif protein 14 MGC15912 11q13.1 hypothetical protein MGC15912 RBM4 11q13 RNA binding motif protein 4 MGC10871 11q13 hypothetical protein similar to RNA-binding protein lark SPTBN2 11q13 spectrin, beta, non-erythrocytic 2 FLJ22531 11q13.1 hypothetical protein FLJ22531 RCE1 11q13 RCE1 homolog, prenyl protein protease (S. cerevisiae) PC 11q13.3 pyruvate carboxylase MGC3103 LOC254439 11q13.1 hypothetical gene supported by AK026328 SYT12 11q12.2 synaptotagmin XII ARHD 11q14.3 ras homolog gene family, member D FBXL11 11q12.2 F-box and leucine-rich repeat protein 11
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    8 - 1485 - 225 - 2003 14504265
    8 - 1489 - 329 - 2003 14504265
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Reproductivefemale systemovary   
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • HXXE sequence characteristic of the M14 family of metalloproteinases
  • a His residue,involved in metal binding
  • a Glu catalytic residue near the C terminus
  • conjugated MetalloP
    HOMOLOGY
    interspecies homolog to yeast S.cerevisiae Rce1p,CAAX-prenyl protein protease
    Homologene
    FAMILY
  • peptidase U48 family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    text
  • integral membrane protein
  • with USP17 co-localize at the endoplasmic reticulum
  • basic FUNCTION
  • catalyzing the the first step of the proteolytic processing of farnesylated protein converting enzyme 2, including Ras
  • has important functional interaction with the P2' and P3' substrate positions in addition to the farnesylated cysteine at the scissile bond site
  • required for lamin B1 endoproteolysis, and involved in integrity of the nuclear lamina
  • promotes a proteolytic activity that is required for the maturation of RAS, and certain other proteins whose precursors bear a C-terminal CAAX tetrapeptide motif
  • involved in the cleavage of the CAAX motif of RAS, motif that are essential for its proper membrane localization and function
  • cysteine protease, but has also metalloprotease-like activity and loss of RCE1 blocks proper RAS localization and impedes cell growth and transformation
  • essential for the intracellular trafficking of PDE6A,PDE6B, PDE6C, PDE6D and survival of photoreceptor cells
  • required for the proper function of photoreceptor cells
  • not needed for the development of retinal neurons but is specifically required for the maintenance of photoreceptor cells
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • multimeric rod phosphodiesterase 6 (PDE6), is a prenylated protein and RCE1 substrate
  • cell & other
    REGULATION
    repressed by USP17
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    cancer  
    peptidomimetic inhibitors of RCE1 may be useful in treatment of cancer
    immunologyinflammatory 
    peptidomimetic inhibitors of RCE1 may be useful in treatment of inflammatory disease
    ANIMAL & CELL MODELS
    hearts from heart-specific Rce1 knockout mice manifested reduced levels of both the Rce1 mRNA and CAAX endoprotease activity, and the hearts manifested an accumulation of CAAX protein substrates; heart-specific Rce1 knockout mice had a dilated cardiomyopathy