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FLASH GENE
Symbol MOGS contributors: mct/npt - updated : 25-02-2016
HGNC name mannosyl-oligosaccharide glycosidase
HGNC id 24862
Corresponding disease
CDG2B congenital disorder of glycosylation, type 2B
Location 2p13.1      Physical location : 74.688.184 - 74.692.537
Genatlas name glucosidase I
Synonym name
  • processing alpha-glucosidase 1
  • mannosyl-oligosaccharide glucosidase
  • Synonym symbol(s) G1NLG, PAG1, GCS1, CDG2B, CWH41, DER7
    EC.number 3.2.1.106
    DNA
    TYPE functioning gene
    STRUCTURE 4.35 kb     4 Exon(s)
    text structure exon-4 encoding for the large 64-kDa catalytic domain of the enzyme
    MAPPING cloned Y linked N status provisional
    Map pter - D2S1726E - D2S1691E - GCS1 - D2S1537E - D2S1577E - cen
    regionally located in the PARK3 region
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    4 - 2910 91 837 - Hardt (2003)
    5 - 2584 - 731 - -
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveintestinesmall intestine  highly
    Reproductivefemale systemovary  highly
     female systemuteruscervix highly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • (Arg)3 within the N-terminal domain contains ER targeting information but not required absolutely for ER localization
  • HOMOLOGY
    Homologene
    FAMILY
  • glycosyl hydrolase 63 family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,lumen
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    basic FUNCTION
  • removing specifically distal alpha1-2 linked glucose from the Glc (3), Man (9), GlcNAc (2) precursor after its en bloc transfer from dolichyl diphosphate to a nascent polypeptide chain in the endoplasmic reticulum
  • cleaves the distal alpha-1,2-linked glucose residue from the Glc(3)-Man(9)-GlcNAc(2) oligosaccharide precursor
  • first enzyme in the processing pathway of N-linked oligosaccharide
  • is involved in the trimming of N-glycans
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism carbohydrate
    signaling
    first step in the N-linked oligosaccharide processing pathway
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) CDG2B
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS