Table 2 ADAMTS knockout and mutant mouse phenotypes

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]