Table 2.

ADAMTS knockout and mutant mouse phenotypes

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]