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Table 2 ADAMTS knockout and mutant mouse phenotypes

From: The ADAMTS (A Disintegrin and Metalloproteinase with Thrombospondin motifs) family

Gene Phenotype of gene knockout or mutant mice Reference(s)
Adamts1 Growth retardation, adipose tissue malformation [91]
  Impaired fertility with defective ovulation [153]
  Severe kidney abnormalities: enlarged renal calices with fibrosis leading to obstruction of uteropelvic junction; abnormal adrenal medullary architecture with no formation of capillaries [92] [148]
[150]
  Defective follicular development during ovulation, delay in development of ovarian lymphatic vessels [94, 95]
  Impaired skin wound healing; effects on keratinocyte and fibroblast migration [98]
  No defects in aggrecan turnover in vivo or in vitro [145]
  Reduced tumorigenesis and metastasis in PyMT mammary cancer, with increased apoptosis [117]
  Defective myocardial morphogenesis [96]
  Selective decline in synaptic protein levels in frontal cortex of female Adamts1−/− mice [58]
Adamts2 Fragile skin at 1–2 months postnatal; male sterility [144]
  Widespread defects in procollagen III processing; abnormal lungs [142]
  Reduced extent and stability of carbon tetrachloride-induced hepatic fibrosis [141]
Adamts4 No phenotype unchallenged [69]
  Perinatal lethality, exacerbation of renal phenotype in Adamts1−/−;Adamts4−/− double knockout mice [138]
Adamts5 No phenotype unchallenged. Protection in surgery-induced osteoarthritis and antigen-induced arthritis models [69]
[68]
  Adamts4−/−;Adamts5−/− double knockout mice phenotypically normal; osteoarthritis phenotype same as Adamts5−/− mice [146]
  Blockade of fibrosis and accumulation of aggrecan in joints in the DMM and TTR models of osteoarthritis [143]
[139]
  Reduced changes in subchondral bone in DMM model of osteoarthritis [152]
  Altered biomechanical properties of tendon [81]
  Cardiac valve defects resembling myxomatous valve disease; rescued in versican (Vcan) heterozygotic animals [147]
  Partial reduction of interdigital web regression [151]
  Impaired dermal repair in excisional skin wound healing; aggrecan accumulation, altered transforming growth factor β (TGFβ) signaling [140]
  Dermal fibroblasts have myofibroblastic phenotype showing increased contractility in three-dimensional collagen gels, rescued in versican (Vcan) heterozygotic animals  
Adamts9 Embryonic lethal at E7.5 days post coitum [83]
  Partial reduction of interdigital web regression, enhanced in Adamts5−/−;Adamts9−/+; bt/bt mice [82]
  Abnormal cardiac development in Adamts9+/− mice [80]
Adamts12 No phenotype unchallenged; Elevated tumor growth and angiogenesis [104]
  Exacerbated inflammation and airway dysfunction in allergen-induced airways disease [149]
  More severe inflammation and delayed recovery following colitis, endotoxic sepsis and pancreatitis induction [130]
Adamts13 Little phenotype unchallenged; loss of ADAMTS13 is pro-thrombotic but insufficient to generate thrombotic thromboscytopenic purpura [137]
Adamts20 Mutations in Adamts20 are found in belted (bt) mice, causing white spotting in the torso due to defective melanoblast survival [83]
  Partial reduction of interdigital web regression, enhanced in Adamts5−/−;Adamts9−/+; bt/bt mice [82]
  Adamts9+/−;bt/bt mice have cleft palate [86]