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Symbol EDEM1 contributors: mct - updated : 08-07-2014
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
TYPE functioning gene
STRUCTURE 32.29 kb     12 Exon(s)
MAPPING cloned Y linked N status provisional
Physical map
CNTN6 3p26-p25 contactin 6 LOC391504 3 similar to 60S ribosomal protein L23a LOC391505 3 similar to 60S ribosomal protein L21 CNTN4 3p26.2 contactin 4 IL5RA 3p26-p25 interleukin 5 receptor, alpha TRNT1 3p25.1 tRNA nucleotidyl transferase, CCA-adding, 1 LOC51185 3p26.2 protein x 0001 LRRN1 3p26.2 leucine rich repeat neuronal 1 SETMAR 3p26.1 SET domain and mariner transposase fusion gene SUMF1 3p26.2 SET domain and mariner transposase fusion gene MRPS10P2 3p26 mitochondrial ribosomal protein S10 pseudogene 2 ITPR1 3p26-p25 inositol 1,4,5-triphosphate receptor, type 1 BHLHB2 3p26.1-p25.3 basic helix-loop-helix domain containing, class B, 2 LOC391506 3 similar to autoantigen NOR-90 FLJ10702 EDEM1 3p26.1 ER degradation enhancer, mannosidase alpha-like 1 MRPS35P1 3p25.3 mitochondrial ribosomal protein S35 pseudogene 1 MRPS36P1 3p25.3 mitochondrial ribosomal protein S36 pseudogene GRM7 3p26.1-p25.2 glutamate receptor, metabotropic 7 LOC389092 3 LOC389092 LMCD1 3p26-p24 LIM and cysteine-rich domains 1 LOH3CR2A 3 loss of heterozygosity, 3, chromosomal region 2, gene A LOC51066 3p25.3 fls485 LOC391507 3 similar to seven transmembrane helix receptor CAV3 3p25 caveolin 3 OXTR 3p25 oxytocin receptor RAD18 3p25-p24 RAD18 homolog (S. cerevisiae) SRGAP2 3p25.3 SLIT-ROBO Rho GTPase activating protein 2 LOC391508 3 similar to Phosphoglycerate mutase 1 (Phosphoglycerate mutase isozyme B) (PGAM-B) (BPG-dependent PGAM 1) DKFZP434F091 3p25.3 DKFZP434F091 protein
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
12 - 6162 - 657 - 2006 16987498
Type widely
   expressed in (based on citations)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Lymphoid/Immunelymph node   highly
 thymus   highly
Reproductivefemale systemuteruscervix highly
Visualeyeretina    Homo sapiens
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Blood / hematopoieticbone marrow  highly
SystemCellPubmedSpeciesStageRna symbol
Visualrod photoreceptor Homo sapiens
cell lineage
cell lines
  • 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)
    interspecies homolog to murine Edem1
    homolog to Htm1p
    intraspecies paralog to EDEM2, EDEM3
    FAMILY glycosyl hydrolase family 47
    CATEGORY regulatory
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,endoplasmic reticulum
  • 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
    metabolism carbohydrate
    a component part of supramolecular functional ERAD complex, comprising EDEM1, DNAJC10, and HSPA5
    small molecule
  • 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
  • cell & other
    induced by various type of ER stress