Gene Symbol: Edem1
Description: ER degradation enhancer, mannosidase alpha-like 1
Alias: A130059K23Rik, EDEM, mKIAA0212, ER degradation-enhancing alpha-mannosidase-like protein 1, ER degradation ER degradation enhancer, mannosidase alpha-like 1, ER degradation-enhancing alpha-mannosidase-like 1
Species: mouse
Products:     Edem1

Top Publications

  1. Oda Y, Hosokawa N, Wada I, Nagata K. EDEM as an acceptor of terminally misfolded glycoproteins released from calnexin. Science. 2003;299:1394-7 pubmed
    ..b>EDEM, a postulated Man8B-binding protein, accelerates the degradation of misfolded proteins in the ER...
  2. Hosokawa N, Wada I, Hasegawa K, Yorihuzi T, Tremblay L, Herscovics A, et al. A novel ER alpha-mannosidase-like protein accelerates ER-associated degradation. EMBO Rep. 2001;2:415-22 pubmed
    ..We report the cDNA cloning of Edem, a mouse gene encoding a putative type II ER transmembrane protein...
  3. Cali T, Galli C, Olivari S, Molinari M. Segregation and rapid turnover of EDEM1 by an autophagy-like mechanism modulates standard ERAD and folding activities. Biochem Biophys Res Commun. 2008;371:405-10 pubmed publisher
    b>EDEM1 is a crucial regulator of endoplasmic reticulum (ER)-associated degradation (ERAD) that extracts non-native glycopolypeptides from the calnexin chaperone system...
  4. Hosokawa N, Tremblay L, Sleno B, Kamiya Y, Wada I, Nagata K, et al. EDEM1 accelerates the trimming of alpha1,2-linked mannose on the C branch of N-glycans. Glycobiology. 2010;20:567-75 pubmed publisher
    ..Herein, we report that overexpression of EDEM1 produces Glc(1)Man(8)GlcNAc(2) isomer C on terminally misfolded null Hong Kong alpha1-antitrypsin (NHK) in vivo...
  5. Molinari M, Calanca V, Galli C, Lucca P, Paganetti P. Role of EDEM in the release of misfolded glycoproteins from the calnexin cycle. Science. 2003;299:1397-400 pubmed
    ..Here the alpha-mannosidase I-like protein EDEM was shown to extract misfolded glycoproteins, but not glycoproteins undergoing productive folding, from the ..
  6. S omi ska Wojew dzka M, Pawlik A, Soko owska I, Antoniewicz J, W grzyn G, Sandvig K. The role of EDEM2 compared with EDEM1 in ricin transport from the endoplasmic reticulum to the cytosol. Biochem J. 2014;457:485-96 pubmed publisher
    b>EDEM1 [ER (endoplasmic reticulum)-degradation-enhancing ?-mannosidase I-like protein 1] and EDEM2 are crucial regulators of ERAD (ER-associated degradation) that extracts non-native glycoproteins from the calnexin chaperone system...
  7. Iwawaki T, Akai R, Yamanaka S, Kohno K. Function of IRE1 alpha in the placenta is essential for placental development and embryonic viability. Proc Natl Acad Sci U S A. 2009;106:16657-62 pubmed publisher
    ..These findings reveal that IRE1alpha plays an essential function in extraembryonic tissues and highlight the relationship of physiological ER stress and angiogenesis in the placenta during pregnancy in mammals. ..
  8. Marin M, Ghenea S, Spiridon L, Chiritoiu G, Petrescu A, Petrescu S. Tyrosinase degradation is prevented when EDEM1 lacks the intrinsically disordered region. PLoS ONE. 2012;7:e42998 pubmed publisher
    b>EDEM1 is a mannosidase-like protein that recruits misfolded glycoproteins from the calnexin/calreticulin folding cycle to downstream endoplasmic reticulum associated degradation (ERAD) pathway...
  9. Zhang B, Zheng C, Zhu M, Tao J, Vasievich M, Baines A, et al. Mice deficient in LMAN1 exhibit FV and FVIII deficiencies and liver accumulation of ?1-antitrypsin. Blood. 2011;118:3384-91 pubmed publisher

More Information


  1. Kaneko K, Xu P, Cordonier E, Chen S, Ng A, Xu Y, et al. Neuronal Rap1 Regulates Energy Balance, Glucose Homeostasis, and Leptin Actions. Cell Rep. 2016;16:3003-3015 pubmed publisher
    ..Thus, neuronal Rap1 critically regulates leptin sensitivity and mediates HFD-induced obesity and hypothalamic pathology and may represent a potential therapeutic target for obesity treatment. ..
  2. Sharma M, Bhattacharyya S, Nain M, Kaur M, Sood V, Gupta V, et al. Japanese encephalitis virus replication is negatively regulated by autophagy and occurs on LC3-I- and EDEM1-containing membranes. Autophagy. 2014;10:1637-51 pubmed publisher
    ..replication complexes furthermore show association with a marker of the ER-associated degradation (ERAD) pathway, EDEM1 (ER degradation enhancer, mannosidase ?-like 1)...
  3. Tao J, Zhu M, Wang H, Afelik S, Vasievich M, Chen X, et al. SEC23B is required for the maintenance of murine professional secretory tissues. Proc Natl Acad Sci U S A. 2012;109:E2001-9 pubmed publisher
  4. Posey K, Coustry F, Veerisetty A, Liu P, Alcorn J, Hecht J. Chop (Ddit3) is essential for D469del-COMP retention and cell death in chondrocytes in an inducible transgenic mouse model of pseudoachondroplasia. Am J Pathol. 2012;180:727-37 pubmed publisher
    ..Taken together, these results suggest that there may be an optimal window before the induction of significant D469del-COMP retention during which endoplasmic reticulum stress could be targeted. ..
  5. Francisco A, Singh R, Li S, Vani A, Yang L, Munroe R, et al. Deficiency of suppressor enhancer Lin12 1 like (SEL1L) in mice leads to systemic endoplasmic reticulum stress and embryonic lethality. J Biol Chem. 2010;285:13694-703 pubmed publisher
    ..Taken together, our results demonstrate an essential role for SEL1L in protein quality control during mouse embryonic development. ..
  6. Eriksson K, Vago R, Calanca V, Galli C, Paganetti P, Molinari M. EDEM contributes to maintenance of protein folding efficiency and secretory capacity. J Biol Chem. 2004;279:44600-5 pubmed
    ..caused by lack of adaptation of the intralumenal level of ER degradation-enhancing alpha-mannosidase-like protein (EDEM) to an increase in the ER cargo load...
  7. Hagiwara M, Maegawa K, Suzuki M, Ushioda R, Araki K, Matsumoto Y, et al. Structural basis of an ERAD pathway mediated by the ER-resident protein disulfide reductase ERdj5. Mol Cell. 2011;41:432-44 pubmed publisher
    ..protein that accelerates ERAD by reducing incorrect disulfide bonds in misfolded glycoproteins recognized by EDEM1. We here solved the crystal structure of full-length ERdj5, thereby revealing that ERdj5 contains the N-terminal J ..
  8. Shenkman M, Groisman B, Ron E, Avezov E, Hendershot L, Lederkremer G. A shared endoplasmic reticulum-associated degradation pathway involving the EDEM1 protein for glycosylated and nonglycosylated proteins. J Biol Chem. 2013;288:2167-78 pubmed publisher
    ..the uncleaved precursor of asialoglycoprotein receptor H2a, its nonglycosylated mutant, makes use of calnexin, EDEM1, and HRD1, but only glycosylated H2a is a substrate for the cytosolic SCF(Fbs2) E3 ubiquitin ligase with lectin ..
  9. Slominska Wojewodzka M, Gregers T, Walchli S, Sandvig K. EDEM is involved in retrotranslocation of ricin from the endoplasmic reticulum to the cytosol. Mol Biol Cell. 2006;17:1664-75 pubmed
    ..The ER degradation enhancing alpha-mannosidase I-like protein, EDEM, is responsible for directing aberrant proteins for ER-associated protein degradation...
  10. Zhu M, Tao J, Vasievich M, Wei W, Zhu G, Khoriaty R, et al. Neural tube opening and abnormal extraembryonic membrane development in SEC23A deficient mice. Sci Rep. 2015;5:15471 pubmed publisher
    ..Our results suggest that mammalian SEC23A and SEC23B transport overlapping yet distinct spectra of cargo in vivo. ..
  11. Gnann A, Riordan J, Wolf D. Cystic fibrosis transmembrane conductance regulator degradation depends on the lectins Htm1p/EDEM and the Cdc48 protein complex in yeast. Mol Biol Cell. 2004;15:4125-35 pubmed
    ..Complementation of the HTM1 deficiency in yeast cells by the mammalian orthologue EDEM underlines the necessity of this lectin for CFTR degradation and highlights the similarity of quality control and ..