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FLASH GENE
Symbol IDUA contributors: mct - updated : 14-11-2012
HGNC name iduronidase, alpha-L-
HGNC id 5391
EXPRESSION
Type
   expressed in (based on citations)
organ(s)
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Connectivebone   
Connectivecartilage  highly
cells
SystemCellPubmedSpeciesStageRna symbol
 digestive
Blood/Hematopoieticleukocyte
Digestivehepatocyte
Nervousneuron
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
conjugated GlycoP
mono polymer monomer
HOMOLOGY
interspecies ortholog to murine Idua
Homologene
FAMILY
  • glycosyl hydrolase 39 family
    • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,organelle,endosome
    intracellular,cytoplasm,organelle,lysosome
    basic FUNCTION
  • iduronidase, alpha-L, hydrolyzing dermatan sulfate, heparan sulfate
  • catalyzing the second step of degradation of glucosaminoglycans (mucopolysaccharides)
  • playing a role in the hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism carbohydrate
    signaling
    disaccharide metabolism
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
    cell & other
    REGULATION
    Other targeted to the lysosome by M6P receptor mediated pathway
    ASSOCIATED DISORDERS
    corresponding disease(s) MPS1
    related resource Mucopolysaccharidosis
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • substantial elevation in heparan sulfate (HS) within the liver and brain of Idua -/- mice and significant changes in HS localization and sulfation patterning with significant increases in N-, 6-O-, and 2-O-sulfation