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FLASH GENE
Symbol ADAMTS2 contributors: mct/npt - updated : 27-10-2006
HGNC name ADAM metallopeptidase with thrombospondin type 1 motif, 2
HGNC id 218
Corresponding disease
EDS7C Ehlers-Danlos syndrome, type VIIC
Location 5q23      Physical location : 178.537.851 - 178.772.431
Synonym name
  • procollagen amino-propeptide processing enzyme
  • a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 2
  • Synonym symbol(s) PCNIP, ADAM-TS2, ADAMTS-3, NPI, PCINP, PCPNI, NPI
    EC.number 3.4.24.14
    DNA
    TYPE functioning gene
    STRUCTURE 231.50 kb     22 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    - - 3661 134 1211 - Reardon
    - - 1701 - 566 - Reardon
  • lacking the C terminal TSP motifs and peptidase activity
  • 11 exons
  • EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   highly
     vesselsaorta  highly
    Lymphoid/Immunethymus   lowly
    Nervousbrain   lowly
    Reproductivefemale systemuterus  highly
    Skin/Tegumentskin   highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Connectiveadipose   
    Connectivebone   
    Connectivedensetendon  
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a signal sequence
  • a propeptide domain
  • a reprolysin-type catalytic domain
  • a disintegrin-like domain
  • four thrombospondin type 1 (TSP1) module
  • a cysteine rich domain
  • a spacer domain without cysteine residues
  • terminal TSP-like modules
  • conjugated MetalloP
    HOMOLOGY
    interspecies ortholog to murine Adamts2
    Homologene
    FAMILY
  • ADAMTS (a disintegrin and metalloproteinase with thrombospondin motif) protein family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION extracellular
    text extracellular matrix
    basic FUNCTION
  • a disintegrin-like and metalloprotease, cell surface adhesion protein, procollagen I/II aminopeptide processing enzyme
  • extracellular metalloproteinase responsible for cleaving the N-propeptides of procollagens I-III
  • cleaving the propeptides of type I and II collagen prior to fibril assembly and may be playing a role in development that is independent of its role in collagen biosynthesis
  • CELLULAR PROCESS protein, post translation
    protein, degradation
    PHYSIOLOGICAL PROCESS development
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule metal binding,
  • Zn2+
  • protein
    cell & other
    REGULATION
    inhibited by TIMP3 (inhibit procollagen processing by cells)
    ASSOCIATED DISORDERS
    corresponding disease(s) EDS7C
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS