Table 2 Summary of p107-deficient mouse models and their phenotypes.

p107 ^-/- (Balb/c)

Germline

Viable

Ectopic myeloid hyperplasia in the spleen and liver, severe postnatal growth deficiency, fibroblasts and myoblasts have increased cell cycle kinetics, decreased white adipose tissue.

[99, 100]

p107 ^-/- (mixed)

Germline

Viable

No gross abnormalities, expanded neural progenitor pool in the embryonic and adult brain.

[105, 108]

Rb ^+/- ;p107 ^-/-

Germline

Viable

Pituitary tumors, reduced viability and growth retardation after birth until ~3 months of age, vaginal atresia (females).

[50]

Rb ^+/- ;p107 ^-/-

Chimera

Viable

Pituitary glad tumors, adenocarcinoma of the caecum, osteosarcoma, lymphosarcoma, occasional retinal dysplasia but no retinoblastoma.

[130]

Rb ^-/- ;p107 ^-/-

Chimera

Viable

Retinoblastoma development between 1 - 3 months of age, adult mice obtained at low frequency, apoptosis in the retina.

[130]

Rb ^-/- ;p107 ^-/-

Germline

Lethal E11.5

Accelerated apoptosis in the liver and CNS.

[97]

Mox2Cre;Rb ^lox/lox ;p107 ^-/-

Conditional (embryo)

Lethal E13.5-E14.5

Hyperproliferation of the CNS, lens, blood vessel endothelial cells. Double-outlet right ventricle (DORV) heart defect.

[142]

p107 ^-/- ;p130 ^-/-

Germline

Birth

Hyperproliferation of chondrocytes, defective endochondral bone development, increased epidermal proliferation, decreased number of hair follicles, developmental delay in whisker, hair, and tooth formation.

[101, 102]

Rb ^-/- ;p107 ^-/- ;hIRBPp53DD

Chimera

Viable

Retinoblastoma.

[44]

SPC-rtTA;tetCre;Rb ^lox/lox ;p107 ^-/-

Conditional (lung)

Viable

~70% of mice develop lung adenoma or adenocarcinoma.

[126]

K14Cre;Rb ^lox/lox ;p107 ^-/-

Conditional (skin)

Viable

Spontaneous squamous cell carcinomas.

[49]

NesCre;Rb ^lox/lox ;p107 ^-/-

Conditional (retina)

Viable

Retinal dysplasia, high levels of apoptosis in the retina.

[129]

Chx10Cre;Rb ^lox/lox ;p107 ^-/-

Conditional (retina)

Viable

Unilateral retinoblastoma, 60% penetrant, delayed onset compared to Rb/p53/p107.

[132]

Chx10Cre;Rb ^lox/lox ;p53 ^lox/lox ; p107 ^-/-

Conditional (retina)

Viable

Aggressive bilateral retinoblastoma, 100% penetrant.

[132, 141]

Chx10Cre;Rb ^lox/lox ;p130 ^-/- ;p107 ^+/-

Conditional (retina)

Viable

Differentiated horizontal neurons of the Inner Nuclear Layer re-enter the cell cycle and form metastatic retinoblastoma.

[133]

Pax6α Cre;Rb ^lox/lox ;p107 ^-/-

Conditional (retina)

Viable

Unilateral retinoblastoma, 60% penetrant, delayed onset compared to Rb/p130.

[131, 143]

En2Cre;Rb ^lox/lox ;p107 ^-/-

Conditional (dorsal mid-hindbrain)

Viable

Ataxia between P15 and P20, disorganized cerebellar architecture, shrunken dendritic arborization, laminar defects, hyperproliferation of granule cell precursors, and granule cell death upon differentiation.

[113]

p107 ^-/- ;p27 ^{D 51/D 51}

Germline

Viable

Chondrocyte hyperproliferation, defective chondrocyte maturation, defective endochondral bone formation.

[103]

Hes1 ^-/- ;p107 ^-/-

Germline

Lethal E12.5

Embryonic lethality due to null Hes1 mutation, restoration of normal numbers of neural precursors in embryos and adults.

[108]