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FLASH GENE
Symbol IMPG1 contributors: /mct - updated : 17-10-2019
HGNC name interphotoreceptor matrix proteoglycan 1
HGNC id 6055
EXPRESSION
Type restricted
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestiveliver    
Nervousbrain    
Visualeye     Homo sapiens
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Muscularstriatumskeletal  
Nervousperipherous   
cells
SystemCellPubmedSpeciesStageRna symbol
Nervousoptic nerve
Visualcone photoreceptor Homo sapiens
Visualrod photoreceptor Homo sapiens
cell lineage
cell lines
fluid/secretion
at STAGE
physiological period fetal
Text eye, anterior segment,retina foveal macular,optic nerve
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • functional hyaluronan binding domain and not etablished glycosaminoglycan attachment site
  • three sites for N glycosylation
  • two SEA domains (after sea urchin sperm protein, enterokinase, and agrin), a highly conserved structure composed of four anti-parallel beta sheets surrounded by alpha helices
  • a central mucin-like domain
  • an EGF-like domain in the C-terminal region
  • conjugated GlycoP , Other
    HOMOLOGY
    Homologene
    FAMILY
  • extracellular matrix glycoconjugates family
  • CATEGORY structural protein
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    text photoreceptor layer including IPM, cone pedicles, rod spherules
    basic FUNCTION
  • participating in retinal adhesion and in maintaining photoreceptor viability
  • photoreceptor cell-associated chondroitin-sulfate proteoglycan
  • important role for IMPG1 and CD44 in the development of photoreceptors and IPM (interphotoreceptor matrix) formation during human retinogenesis
  • IMPG1 and IMPG2 have distinct pattern distributions in the IPM, and both cones and rods are surrounded by IMPG proteoglycans
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • constituent of interphotoreceptor matrix, associated with cones and rods
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • when IMPG2 is absent, IMPG1 cannot integrate into the interphotoreceptor matrix (IPM), leading to abnormal proteoglycan accumulation and visual deficits )
  • cell & other
  • with IMPG2, bind to hyaluronan, a glycosaminoglycan molecule that is essential for the organization of the interphotoreceptor matrix (Bandah-Rozenfeld 2010)
  • REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) BCAMD1 , VMD4
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • mice lacking both Impg1 and Impg2, regardless of sex, showed normal retinal structure and function, demonstrating that the aberrant Impg1 distribution is the main cause of the visual alterations observed in the absence of Impg2 )