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FLASH GENE
Symbol EDEM1 contributors: mct - updated : 08-07-2016
HGNC name ER degradation enhancer, mannosidase alpha-like 1
HGNC id 18967
Location 3p26.1      Physical location : 5.229.358 - 5.261.649
Synonym name ER degradation enhancing alpha mannosidase-like
Synonym symbol(s) KIAA0212, EDEM, FLJ51559, FLJ51560
DNA
TYPE functioning gene
STRUCTURE 32.29 kb     12 Exon(s)
MAPPING cloned Y linked N status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
12 - 6162 - 657 - 2006 16987498
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Lymphoid/Immunelymph node   highly
 thymus   highly
Reproductivefemale systemuteruscervix highly
Visualeyeretina    Homo sapiens
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Blood / hematopoieticbone marrow  highly
cells
SystemCellPubmedSpeciesStageRna symbol
Visualrod photoreceptor Homo sapiens
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • five N-linked glycosylation consensus sites
  • one of the five glycosylation sites is located near the C terminus of EDEM1 (Asn-624)
  • a mannosidase-like domain
  • a signal sequence (cleavage of this signal functionally regulated the association of EDEM1-soluble and membrane-integrated isoforms with distinct ERAD machinery and substrates)
    • HOMOLOGY
    interspecies homolog to murine Edem1
    homolog to Htm1p
    intraspecies paralog to EDEM2, EDEM3
    Homologene
    FAMILY glycosyl hydrolase family 47
    CATEGORY regulatory
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    text
  • initially reported to be a membrane protein, but more recent results have indicated that it is also a soluble protein
    • basic FUNCTION
  • involved in ER-associated degradation (ERAD)
  • accelerate degradation of misfolded glycoproteins of the calnexin cycle
  • lacks mannosidase activity
  • putative mannose-binding protein, targeting misfolded glycoproteins for ERAD, and sequestered in buds that form along cisternae of the rough ER at regions outside of the transitional ER
  • ER stress-induced protein that regulates disposal of folding-defective glycoproteins and has been described as a mannose-binding lectin
  • can enhance the release of terminally misfolded glycoproteins from the calnexin chaperone system
  • its activity trims mannose from the C branch of N-glycans
  • is involved in trimming of mannose residues from precursor N-glycans during glycoprotein ER quality control
  • mannosidase-like protein that recruits misfolded glycoproteins from the calnexin/calreticulin folding cycle to downstream endoplasmic reticulum associated degradation (ERAD) pathway
  • accelerating tyrosinase degradation by EDEM1 overexpression may lead to an efficient antigen presentation and enhanced elimination of melanoma cells
  • implicated in the targeting of the nonglycoprotein ricin toxin for retrotranslocation
  • EDEM1 and EDEM2 are crucial regulators of ERAD (ER-associated degradation) that extracts non-native glycoproteins from the calnexin chaperone system
    • CELLULAR PROCESS protein, degradation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism carbohydrate
    signaling
    a component part of supramolecular functional ERAD complex, comprising EDEM1, DNAJC10, and HSPA5
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacts with calnexin
  • binding with DNAJC10
  • associates with a number of ER proteins and ER-associated degradation (ERAD) substrates
  • DERL2 and DERL3 provide the missing link between EDEM1 and VCP in the process of degrading misfolded glycoproteins
  • binds nonnative proteins and uses its mannosidase-like domain to target aberrant proteins to the ER membrane dislocation and ubiquitination complex containing SEL1L
  • novel chaperone of rod opsin
  • interacting with ERLEC1 (mannose trimming is required for delivery of a glycoprotein from EDEM1 to ERLEC1 and to late endoplasmic reticulum-associated degradation steps)
  • DNAJC10 interacts with EDEM1 and reduces EDEM1-recruited substrates, leading to their facilitated degradation
  • membrane form of EDEM1 (NA-EDEM1) associated most significantly with the membrane protein SEL1L
  • associates through a region outside of its mannosidase-like domain with the nonglycosylated proteins
  • central player in glycoprotein ERAD, can bind a glycoprotein ERAD substrate in a glycan-independent manner
  • EDEM1, EDEM2 associate with oxidoreductases, protein disulfide isomerase, and especially TXNDC11, enhancing mannosidase activity on glycoproteins but not on free N-glycans
    • cell & other
    REGULATION
    induced by various type of ER stress
    ASSOCIATED DISORDERS
    ANIMAL & CELL MODELS