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Table 2 ADAMTS knockout and mutant mouse phenotypes

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


Phenotype of gene knockout or mutant mice



Growth retardation, adipose tissue malformation



Impaired fertility with defective ovulation



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]



Defective follicular development during ovulation, delay in development of ovarian lymphatic vessels

[94, 95]


Impaired skin wound healing; effects on keratinocyte and fibroblast migration



No defects in aggrecan turnover in vivo or in vitro



Reduced tumorigenesis and metastasis in PyMT mammary cancer, with increased apoptosis



Defective myocardial morphogenesis



Selective decline in synaptic protein levels in frontal cortex of female Adamts1−/− mice



Fragile skin at 1–2 months postnatal; male sterility



Widespread defects in procollagen III processing; abnormal lungs



Reduced extent and stability of carbon tetrachloride-induced hepatic fibrosis



No phenotype unchallenged



Perinatal lethality, exacerbation of renal phenotype in Adamts1−/−;Adamts4−/− double knockout mice



No phenotype unchallenged. Protection in surgery-induced osteoarthritis and antigen-induced arthritis models




Adamts4−/−;Adamts5−/− double knockout mice phenotypically normal; osteoarthritis phenotype same as Adamts5−/− mice



Blockade of fibrosis and accumulation of aggrecan in joints in the DMM and TTR models of osteoarthritis




Reduced changes in subchondral bone in DMM model of osteoarthritis



Altered biomechanical properties of tendon



Cardiac valve defects resembling myxomatous valve disease; rescued in versican (Vcan) heterozygotic animals



Partial reduction of interdigital web regression



Impaired dermal repair in excisional skin wound healing; aggrecan accumulation, altered transforming growth factor β (TGFβ) signaling



Dermal fibroblasts have myofibroblastic phenotype showing increased contractility in three-dimensional collagen gels, rescued in versican (Vcan) heterozygotic animals



Embryonic lethal at E7.5 days post coitum



Partial reduction of interdigital web regression, enhanced in Adamts5−/−;Adamts9−/+; bt/bt mice



Abnormal cardiac development in Adamts9+/− mice



No phenotype unchallenged; Elevated tumor growth and angiogenesis



Exacerbated inflammation and airway dysfunction in allergen-induced airways disease



More severe inflammation and delayed recovery following colitis, endotoxic sepsis and pancreatitis induction



Little phenotype unchallenged; loss of ADAMTS13 is pro-thrombotic but insufficient to generate thrombotic thromboscytopenic purpura



Mutations in Adamts20 are found in belted (bt) mice, causing white spotting in the torso due to defective melanoblast survival



Partial reduction of interdigital web regression, enhanced in Adamts5−/−;Adamts9−/+; bt/bt mice



Adamts9+/−;bt/bt mice have cleft palate