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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Zp3-cre)93Knw
transgene insertion 93, Barbara B Knowles
MGI:2176187
Summary 23 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Ptentm1Hwu/Ptentm1Hwu
Tg(Zp3-cre)93Knw/0
B6.Cg-Ptentm1Hwu Tg(Zp3-cre)93Knw MGI:4882031
cn2
Tg(Zp3-cre)93Knw/0
Zfp57tm1Xjli/Zfp57tm1Xjli
either: (involves: 129S6/SvEvTac * C57BL/6J) or (involves: 129S6/SvEvTac * Black Swiss * C57BL/6J) MGI:3826998
cn3
Tg(Zp3-cre)93Knw/0
Ttktm1Katj/Ttktm1Katj
involves: 129 * C57BL/6 * C57BL/6J MGI:5056318
cn4
Rnf2tm2Mvl/Rnf2tm2Mvl
Tg(Zp3-cre)93Knw/?
involves: 129 * C57BL/6J MGI:3805327
cn5
Ezh2tm1Sho/Ezh2tm1Sho
Tg(Zp3-cre)93Knw/?
involves: 129 * C57BL/6J MGI:3805332
cn6
Pdha1tm1Ptl/Pdha1tm1Ptl
Tg(Zp3-cre)93Knw/0
involves: 129P2/OlaHsd * C57BL/6J MGI:3843840
cn7
Ctnnb1tm2Kem/Ctnnb1tm2.1Kem
Tg(Zp3-cre)93Knw/0
involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6J MGI:5440179
cn8
Ctnnb1tm2Kem/Ctnnb1tm2.1Kem
Gt(ROSA)26Sortm1(Ctnnb1)Kem/Gt(ROSA)26Sor+
Tg(Zp3-cre)93Knw/0
involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6J MGI:5440176
cn9
Pdss2tm1.1Dalg/Pdss2tm1.1Dalg
Tg(Zp3-cre)93Knw/?
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 MGI:3805704
cn10
Dicer1tm1.1Mnn/Dicer1tm1.2Mnn
Tg(Zp3-cre)93Knw/0
involves: 129S1/SvImJ * C57BL/6J MGI:3850212
cn11
Hsf1tm1.1Echr/Hsf1tm1.1Echr
Tg(Zp3-cre)93Knw/0
involves: 129S2/SvPas * C57BL/6J MGI:5285188
cn12
Pdpk1tm1Maka/Pdpk1tm1Maka
Tg(Zp3-cre)93Knw/0
involves: 129S4/SvJae * C57BL/6J MGI:5013919
cn13
Pdpk1tm1Maka/Pdpk1tm1Maka
Ptentm1Hwu/Ptentm1Hwu
Tg(Zp3-cre)93Knw/0
involves: 129S4/SvJae * C57BL/6J MGI:5013920
cn14
Nr6a1tm3Coo/Nr6a1tm3Coo
Tg(Zp3-cre)93Knw/0
involves: 129S7/SvEvBrd * C57BL/6 MGI:2674001
cn15
Htr2ctm2Jke/Y
Tg(Zp3-cre)93Knw/0
involves: 129X1/SvJ * C57BL/6J MGI:5569622
cn16
Cdx2tm1.1Aral/Cdx2tm1.2Aral
Tg(Zp3-cre)93Knw/0
involves: 129X1/SvJ * C57BL/6 * SJL MGI:5445747
cn17
Cdx2tm1.1Aral/Cdx2tm1.1Aral
Tg(Zp3-cre)93Knw/0
involves: 129X1/SvJ * C57BL/6 * SJL MGI:5445746
cn18
Cdx2tm1.1Aral/Cdx2tm1Fbe
Tg(Zp3-cre)93Knw/0
involves: 129X1/SvJ * C57BL/6 * SJL MGI:5445751
cn19
Lmnatm4Stw/Lmnatm4Stw
Tg(Zp3-cre)93Knw/0
involves: C57BL/6J MGI:5774437
cn20
Snord116tm1Uta/Snord116+
Tg(Zp3-cre)93Knw/0
involves: C57BL/6J MGI:3774114
cn21
Sox9tm2Crm/Sox9tm2Crm
Tg(Zp3-cre)93Knw/?
involves: C57BL/6J * 129S/SvEv MGI:3719095
cn22
Cenpjtm1d(EUCOMM)Wtsi/Cenpjtm1c(EUCOMM)Wtsi
Tg(Zp3-cre)93Knw/0
involves: C57BL/6J * C57BL/6N * FVB/NJ MGI:5613975
tg23
Tg(Zp3-cre)93Knw/0 C57BL/6-Tg(Zp3-cre)93Knw MGI:3835429


Genotype
MGI:4882031
cn1
Allelic
Composition
Ptentm1Hwu/Ptentm1Hwu
Tg(Zp3-cre)93Knw/0
Genetic
Background
B6.Cg-Ptentm1Hwu Tg(Zp3-cre)93Knw
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptentm1Hwu mutation (3 available); any Pten mutation (41 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
N
• mice exhibit normal follicular development, showing healthy corpora lutea and preovulatory follicles at 16 weeks of age, normal resumption of meiosis, ovulation, fertilization and fertility, although PI3K-Akt signaling is elevated




Genotype
MGI:3826998
cn2
Allelic
Composition
Tg(Zp3-cre)93Knw/0
Zfp57tm1Xjli/Zfp57tm1Xjli
Genetic
Background
either: (involves: 129S6/SvEvTac * C57BL/6J) or (involves: 129S6/SvEvTac * Black Swiss * C57BL/6J)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Zp3-cre)93Knw mutation (2 available)
Zfp57tm1Xjli mutation (0 available); any Zfp57 mutation (34 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cellular
• 50% of homozygous mice from homozygous females are dead by E15.5 and 90% are dead by E17.5

mortality/aging
• 50% of homozygous mice from homozygous females are dead by E15.5 and 90% are dead by E17.5




Genotype
MGI:5056318
cn3
Allelic
Composition
Tg(Zp3-cre)93Knw/0
Ttktm1Katj/Ttktm1Katj
Genetic
Background
involves: 129 * C57BL/6 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Zp3-cre)93Knw mutation (2 available)
Ttktm1Katj mutation (0 available); any Ttk mutation (26 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
• oocytes exhibit accelerated anaphase and polar body extrusion compared with wild-type oocytes
• oocytes exhibit defective spindle assembly checkpoint and premature chromosome segregation compared with wild-type oocytes
• oocytes exhibit defective spindle assembly checkpoint and premature chromosome segregation compared with wild-type oocytes
• in female mice

cellular
• 70% of oocytes are aneuploid, with both hyperploidies and hypoploidies, unlike wild-type oocytes




Genotype
MGI:3805327
cn4
Allelic
Composition
Rnf2tm2Mvl/Rnf2tm2Mvl
Tg(Zp3-cre)93Knw/?
Genetic
Background
involves: 129 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rnf2tm2Mvl mutation (0 available); any Rnf2 mutation (22 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
phenotype not analyzed




Genotype
MGI:3805332
cn5
Allelic
Composition
Ezh2tm1Sho/Ezh2tm1Sho
Tg(Zp3-cre)93Knw/?
Genetic
Background
involves: 129 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ezh2tm1Sho mutation (0 available); any Ezh2 mutation (38 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
phenotype not analyzed




Genotype
MGI:3843840
cn6
Allelic
Composition
Pdha1tm1Ptl/Pdha1tm1Ptl
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdha1tm1Ptl mutation (1 available); any Pdha1 mutation (20 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
• oocytes have fewer polar bodies than in wild-type mice
• fewer oocytes at found at the surrounded nucleolus stage than in wild-type oocytes
• more oocytes in the cumulus-oocyte complexes are at the germinal vesicle or abnormal germinal vesicle breakdown stage than in wild-type mice
• following removal of cumulus cells, a higher proportion of oocytes are at the germinal vesicle or abnormal germinal vesicle breakdown stage compared to similarly treated wild-type oocytes
• oocytes exhibit worsening energy status during meiotic maturation unlike wild-type oocytes
• however, oocyte size is normal
• oocytes have fewer polar bodies than in wild-type mice
• some oocytes exhibit chromatin and spindle abnormalities with or without formation of polar bodies
• oocytes are atypical of any developmental stage unlike wild-type oocytes that are at metaphase of meiosis I or meiosis II
• some oocytes have highly condensed chromatin and absence of spindle structures unlike wild-type oocytes
• some oocytes exhibit chromatin that is eccentrically located with only occasional evidence of discrete chromosomes or clumps of chromatin unlike in wild-type oocytes
• some oocytes exhibit chromatin and spindle abnormalities with or without formation of polar bodies unlike wild-type oocytes
• some oocytes have highly condensed chromatin and absence of spindle structures
• some oocytes exhibit chromatin and spindle abnormalities with or without formation of polar bodies




Genotype
MGI:5440179
cn7
Allelic
Composition
Ctnnb1tm2Kem/Ctnnb1tm2.1Kem
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ctnnb1tm2.1Kem mutation (0 available); any Ctnnb1 mutation (27 available)
Ctnnb1tm2Kem mutation (1 available); any Ctnnb1 mutation (27 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

embryo
• absence of embryonic structures at E8.5




Genotype
MGI:5440176
cn8
Allelic
Composition
Ctnnb1tm2Kem/Ctnnb1tm2.1Kem
Gt(ROSA)26Sortm1(Ctnnb1)Kem/Gt(ROSA)26Sor+
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ctnnb1tm2.1Kem mutation (0 available); any Ctnnb1 mutation (27 available)
Ctnnb1tm2Kem mutation (1 available); any Ctnnb1 mutation (27 available)
Gt(ROSA)26Sortm1(Ctnnb1)Kem mutation (0 available); any Gt(ROSA)26Sor mutation (329 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

embryo
• at E8.5 embryos have a sac-like structure composed of 2 layers where the outer layer is reminiscent of the visceral endoderm and the inner layer has characteristics of a pseudostratified epithelium
• expression analysis indicates failure to gastrulate
• formation of proper embryonic structures is incomplete




Genotype
MGI:3805704
cn9
Allelic
Composition
Pdss2tm1.1Dalg/Pdss2tm1.1Dalg
Tg(Zp3-cre)93Knw/?
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdss2tm1.1Dalg mutation (0 available); any Pdss2 mutation (20 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging




Genotype
MGI:3850212
cn10
Allelic
Composition
Dicer1tm1.1Mnn/Dicer1tm1.2Mnn
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129S1/SvImJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dicer1tm1.1Mnn mutation (0 available); any Dicer1 mutation (52 available)
Dicer1tm1.2Mnn mutation (0 available); any Dicer1 mutation (52 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
• nearly all ovulated oocytes exhibit defects in spindle and scattered chromosomes unlike in wild-type oocytes
• 8 hours post germinal vesicle break-down, oocytes contain abnormal chromosome arrangement and multiple spindles unlike wild-type oocytes
• wild-type germinal vesicles transplanted into oocytes exhibit abnormal meiosis
• however, transplantation of the germinal vesicle into a wild-type oocyte normalizes meiosis
• 8 hours post germinal vesicle break-down, mutant oocytes contain multiple spindles
• despite normal numbers of oocytes, female mice are infertile




Genotype
MGI:5285188
cn11
Allelic
Composition
Hsf1tm1.1Echr/Hsf1tm1.1Echr
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129S2/SvPas * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hsf1tm1.1Echr mutation (0 available); any Hsf1 mutation (7 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
• in oocytes chromosomal length is significantly increased at metaphase I
• significantly blocked in the pro-Mi/MI phase
• persistent activation of the spindle assembly checkpoint




Genotype
MGI:5013919
cn12
Allelic
Composition
Pdpk1tm1Maka/Pdpk1tm1Maka
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129S4/SvJae * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdpk1tm1Maka mutation (0 available); any Pdpk1 mutation (119 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cellular
• 92.5% of two-cell embryos from mutant females mated with wild-type males arrest at the two-cell stage when cultured in vitro compared to 4.2% of wild-type control embryos; heterozygous embryos do not express PDPK1 protein, indicating that this protein is not yet synthesized from the paternal allele in these embryos
• two-cell embryos resulting from mutant female mating to wild-type males exhibit suppression of embryonic genome activation and defective G2-to-M transition of the second mitotic cell cycle

reproductive system
• females are sterile, however follicular development and oocyte maturation are normal
• fertilization is also normal in mutant females, with numbers of two-cell embryos from pregnant females comparable with those from controls




Genotype
MGI:5013920
cn13
Allelic
Composition
Pdpk1tm1Maka/Pdpk1tm1Maka
Ptentm1Hwu/Ptentm1Hwu
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129S4/SvJae * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdpk1tm1Maka mutation (0 available); any Pdpk1 mutation (119 available)
Ptentm1Hwu mutation (3 available); any Pten mutation (41 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
N
• fertility of double mutant females is completely restored from 10-30 weeks of age when mated with wild-type males compared to single conditional homozygous Pdpk1 mutants
• embryos derived from double mutant females mated with wild-type males show normal perimplantation development, indicating rescue of the two-cell arrest and the suppressed embryonic genome activation seen in embryos from single conditional homozygous Pdpk1 mutants




Genotype
MGI:2674001
cn14
Allelic
Composition
Nr6a1tm3Coo/Nr6a1tm3Coo
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nr6a1tm3Coo mutation (0 available); any Nr6a1 mutation (126 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
• 6 of 7 mutant females at diestrus display 1 to 3 follicles containing double oocytes
• double-oocyte follicles are observed at the primary, secondary, and antral stages
• however, ovarian histology is otherwise normal, and no differences in ovary weight or size are observed
• double-oocyte follicles may be derived from a single oocyte, as two nucleus-like structures have been noted in one oocyte
• the expression levels of two oocyte-specific genes, BMP15 and GDF9, are increased >2-fold in mutant ovaries at diestrus (but not at estrus or metestrus) relative to those in control ovaries
• the diestrus phase of the estrous cycle is significantly prolonged
• the average length of the estrous cycle is much longer than that of control littermates, as a result of a prolonged diestrus phase
• no differences are observed at the other stages of the estrous cycle
• female mutants are subfertile, showing a ~50% reduction in the number of litters per month relative to control littermates
• at day 5 after birth, the number of pups per litter is significantly lower than that of control littermates

homeostasis/metabolism
N
• at 2-3 months of age, mutant females exhibit:
• normal cicrulating levels of FSH, LH, testosterone and progesterone at proestrus and estrus
• normal estradiol levels at estrus
• normal LH levels at diestrus
• at diestrus, mutant females display significantly reduced circulating testosterone levels relative control littermates
• at diestrus, mutant females display significantly reduced circulating estradiol levels relative control littermates
• at diestrus, mutant females display significantly reduced circulating FSH levels relative control littermates
• at diestrus, mutant females display significantly reduced circulating progesterone levels relative control littermates

endocrine/exocrine glands
• 6 of 7 mutant females at diestrus display 1 to 3 follicles containing double oocytes
• double-oocyte follicles are observed at the primary, secondary, and antral stages
• however, ovarian histology is otherwise normal, and no differences in ovary weight or size are observed




Genotype
MGI:5569622
cn15
Allelic
Composition
Htr2ctm2Jke/Y
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Htr2ctm2Jke mutation (0 available); any Htr2c mutation (10 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• a high rate of seizure-induced mortality is observed from 6 weeks through 17 weeks
• Background Sensitivity: with further backcrossing to enrich the C57BL/6J background content, the mortality phenotype is almost eliminated

growth/size/body
• on an obesogenic (high fat,high sugar) diet, higher rate of obesity is observed compared to controls

behavior/neurological
• mice exhibit seizure-induced death

homeostasis/metabolism
• on an obesogenic (high fat,high sugar) diet, higher rate of obesity is observed compared to controls
• mice show enhanced hyperglycemia incidence when fed an obesogenic (high fat,high sugar) diet

nervous system
• mice exhibit seizure-induced death




Genotype
MGI:5445747
cn16
Allelic
Composition
Cdx2tm1.1Aral/Cdx2tm1.2Aral
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdx2tm1.1Aral mutation (0 available); any Cdx2 mutation (11 available)
Cdx2tm1.2Aral mutation (0 available); any Cdx2 mutation (11 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
N
• when bred to wild-type males females produce viable offspring indicating maternal expression is not required for development




Genotype
MGI:5445746
cn17
Allelic
Composition
Cdx2tm1.1Aral/Cdx2tm1.1Aral
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdx2tm1.1Aral mutation (0 available); any Cdx2 mutation (11 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
N
• when bred to wild-type males females produce viable offspring indicating maternal expression is not required for development




Genotype
MGI:5445751
cn18
Allelic
Composition
Cdx2tm1.1Aral/Cdx2tm1Fbe
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: 129X1/SvJ * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdx2tm1.1Aral mutation (0 available); any Cdx2 mutation (11 available)
Cdx2tm1Fbe mutation (0 available); any Cdx2 mutation (11 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• reach the blastocyst stage then collapse around implantation

embryo
• reach the blastocyst stage then collapse around implantation




Genotype
MGI:5774437
cn19
Allelic
Composition
Lmnatm4Stw/Lmnatm4Stw
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm4Stw mutation (0 available); any Lmna mutation (65 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice are born at normal Mendelian ratios but survive only to P12-P18

growth/size/body
• by P14, mice of both sexes are significantly smaller than wild-type or heterozygous controls
• by P12-P14, body weight is ~50% that of wild-type or heterozygous controls
• mice exhibit retarded growth from P10 onwards

behavior/neurological
• mice display a stiff walking posture

muscle
• significantly smaller diameter myofibrils in quadriceps muscles
• increased incidence of centrally located nuclei in quadriceps muscles

limbs/digits/tail




Genotype
MGI:3774114
cn20
Allelic
Composition
Snord116tm1Uta/Snord116+
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Snord116tm1Uta mutation (1 available); any Snord116 mutation (2 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• almost all mutants with the paternally inherited deletion survive to adulthood and appear healthy at 18 months

growth/size/body
• at 5 months of age, body lengths of mutant males and females are shorter by around 4 and 3%, respectively, than wild-type mice (differences are statistically significant)
• when fed a high-fat diet for 4 months from 8 weeks of age, mutants carrying the paternally-inherited deletion gain less weight than wild-type littermates, with the differences more pronounced in males
• at birth, pups with the paternally-inherited allele are indistinguishable from normal littermates, but beginning at P2, mutants fail to gain weight as effectively as littermates; by 3 weeks, mutant weights are 60% of wild-type male and female weights
• growth rates appear to normalize after weaning, but weight differences persist to maturity in both genders

behavior/neurological
N
• unlike other mouse models of Prader-Willi syndrome, mutant pups carrying the paternally-inherited deletion show normal milk intake, righting ability, and muscle tone and strenght after birth and during early postnatal period
• in accelerating rotarod trials at 2 and 5 months of age, mice carrying the paternally-inherited deletion display essentially flat learning curves (little improvement) over a 6-day training period, whereas control animals display significant improvements
• when provided with a high-fat diet, both wild-type and mutant animals reduce their total food intake, but both mutant males and females have lower food intake during the 'day-time' phase relative to wild-type animals (total energy intake is similar to wild-type, indicating that mutants compensate for reduced 'day-time' intake by increasing intake during dark-phase)
• at 6 months of age, both males and females carrying the paternally-inherited deletion allele show increases in daily food intake normalized to body weight (22% or 32%, respectively; at 3 months, males show significant hyperphagia relative to wild-type males, but to a lesser degree than at 6 months, while in females carrying the paternally-inherited deletion allele, daily food intake increases do not reach significance
• at 10 months, males and females carrying the paternally-inherited deletion allele display significant hyperphagia with daily food intake increased by 31 and 29% respectively compared to wild-type animals
• 3-4 month old mice with the paternally-inherited deletion exhibit increased anxiety-relatted behavior in elevated plus-maze tests (more entries into and time spent in closed arms of maze relative to wild-type mice)

nervous system
• at P5 and 13, brain weight is only slight decreased (95% and 92%, respectively, of wild-type brain weight)

reproductive system
N
• testis and ovary sizes of mutants carrying the paternally-inherited deletion are proportional to their body size, and histologically normal
• in mutant females with the paternally-inherited deletion, vaginal opening is delayed by 3.7 days

endocrine/exocrine glands
N
• morphology of pituitary gland in mutants is normal

homeostasis/metabolism
• when mice are restricted to 80% of their normal food intake levels, wild-type mice gradually lose weight but animals carrying the paternally-inherited deletion allele are better at maintaining their weight (females are significantly better, but males also show improved weight stability compared to wild-type)
• when fed a high-fat diet for 4 months from 8 weeks of age, mutants carrying the paternally-inherited deletion gain less weight than wild-type littermates, with the differences more pronounced in males
• on a high-fat diet, males carrying the paternally-inherited deletion allele have lower resting blood glucose levels and display a smaller peak level after glucose injecion compared to wild-type males
• when allowed ad libitum food access, adult mice carrying the paternally-inherited deletion allele have ghrelin levels 2.3-fold higher than in wild-type animals
• animals with paternally-inherited mutant allele have 39% of wild-type level at 4 weeks of age, and 57% of wild-type levels at 8 weeks
• mice carrying the paternally-inherited deletion allele have significantly rates of oxygen consumption
• mice carrying the paternally-inherited deletion allele exhibit a higher respiratory exchange ratio than wild-type mice
• on a normal diet, males carrying the paternally-inherited deletion have similar basal blood glucose levels to wild-type but display significantly increased response to insulin injection; mutant males fed a high-fat diet also display significantly increased insulin sensitivity

adipose tissue
• on high-fat and normal chow diets, animals carrying the paternally-inherited deletion allele display decreased fat storage compared to wild-type animals
• when fed a high-fat diet for 4 months, mutants carrying the paternally-inherited deletion allele have significantly lower body fat than wild-type animals (mutant males -6.9%, mutant females -7.2% lower than wild-type)
• 5 month-old mutant males on regular chow diet show an insignificant trend (-3.6%) toward decreased body fat content while females show a significant decrease of 4.2% relative to wild-type; 9-month old animals on a regular chow diet significantly lower body fat content (-9% in mutant males, -5% in females)

digestive/alimentary system
• stomachs in animals receiving paternally-inherited mutant allele are significantly smaller than in wild-type littermates; at P5 and 13, stomachs are 67 and 68% the size of wild-type

liver/biliary system
• size is strikingly reduced in size upon examination at P5 and 13
• at P5, liver weight is 72% of wild-type animals, and at p13, liver weight is only 56% of wild-type

cellular
• the Snord116 cluster is imprinted and the maternal copy is silenced during oogenesis; only inheritance of the paternal allele with the Snord116 cluster deletion produces the growth deficiency and polyphagia phenotypes in mice
• when offspring have maternal inheritance of the deleted allele, they are normal in size and show normal lifespans

Mouse Models of Human Disease
OMIM ID Ref(s)
Prader-Willi Syndrome; PWS 176270 J:131427




Genotype
MGI:3719095
cn21
Allelic
Composition
Sox9tm2Crm/Sox9tm2Crm
Tg(Zp3-cre)93Knw/?
Genetic
Background
involves: C57BL/6J * 129S/SvEv
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sox9tm2Crm mutation (1 available); any Sox9 mutation (14 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• trunk neural crest derivatives are reduced
• at E10.5, fewer neurons and glia are present in the dorsal root ganglia caudal to the forelimb level

embryo
• neural crest cells undergo more apoptosis than in wild-type mice prior to or shortly after commencing migration to the periphery
• at E10.5, apoptotic cells are increased in the dorsal region of the neural tube caudal to the forelimb level
• trunk neural crest derivatives are reduced

cellular
• neural crest cells undergo more apoptosis than in wild-type mice prior to or shortly after commencing migration to the periphery
• at E10.5, apoptotic cells are increased in the dorsal region of the neural tube caudal to the forelimb level




Genotype
MGI:5613975
cn22
Allelic
Composition
Cenpjtm1d(EUCOMM)Wtsi/Cenpjtm1c(EUCOMM)Wtsi
Tg(Zp3-cre)93Knw/0
Genetic
Background
involves: C57BL/6J * C57BL/6N * FVB/NJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cenpjtm1c(EUCOMM)Wtsi mutation (0 available); any Cenpj mutation (17 available)
Cenpjtm1d(EUCOMM)Wtsi mutation (0 available); any Cenpj mutation (17 available)
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

embryo
• indistinguishable from germ line null mice
• arrest by E9.0




Genotype
MGI:3835429
tg23
Allelic
Composition
Tg(Zp3-cre)93Knw/0
Genetic
Background
C57BL/6-Tg(Zp3-cre)93Knw
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Zp3-cre)93Knw mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
N
• females produce an average of six pups per litter





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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
08/17/2016
MGI 6.05
The Jackson Laboratory