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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Usp9xtm1Tuv
targeted mutation 1, David A Tuveson
MGI:5438072
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Krastm4Tyj/Kras+
Tg(Pdx1-cre)6Tuv/0
Usp9xtm1Tuv/Y
involves: 129S4/SvJae * FVB/N MGI:5438090
cn2
Krastm4Tyj/Kras+
Tg(Pdx1-cre)6Tuv/0
Usp9xtm1Tuv/Usp9x+
involves: 129S4/SvJae * FVB/N MGI:5438091
cn3
Usp9xtm1Tuv/Y
Tg(Ddx4-cre)1Dcas/0
involves: FVB MGI:7285921


Genotype
MGI:5438090
cn1
Allelic
Composition
Krastm4Tyj/Kras+
Tg(Pdx1-cre)6Tuv/0
Usp9xtm1Tuv/Y
Genetic
Background
involves: 129S4/SvJae * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Krastm4Tyj mutation (9 available); any Kras mutation (76 available)
Tg(Pdx1-cre)6Tuv mutation (3 available)
Usp9xtm1Tuv mutation (0 available); any Usp9x mutation (24 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• due to local or metastatic pancreatic cancer or aggressive oral papillomas

neoplasm
• aggressive oral papillomas
• within 3 months, mice develop pancreatic intraepithelial neoplasia and microinvasive neoplasms
• in the face and urogenital area at 3 months

integument
• in the face and urogenital area at 3 months

endocrine/exocrine glands
• within 3 months, mice develop pancreatic intraepithelial neoplasia and microinvasive neoplasms

digestive/alimentary system
• aggressive oral papillomas

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
pancreatic ductal adenocarcinoma DOID:3498 J:186717




Genotype
MGI:5438091
cn2
Allelic
Composition
Krastm4Tyj/Kras+
Tg(Pdx1-cre)6Tuv/0
Usp9xtm1Tuv/Usp9x+
Genetic
Background
involves: 129S4/SvJae * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Krastm4Tyj mutation (9 available); any Kras mutation (76 available)
Tg(Pdx1-cre)6Tuv mutation (3 available)
Usp9xtm1Tuv mutation (0 available); any Usp9x mutation (24 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• due to local or metastatic pancreatic cancer or aggressive oral papillomas

neoplasm
• aggressive oral papillomas
• within 3 months, mice develop pancreatic intraepithelial neoplasia and microinvasive neoplasms
• in the face and urogenital area at 3 months

integument
• in the face and urogenital area at 3 months

digestive/alimentary system
• aggressive oral papillomas

endocrine/exocrine glands
• within 3 months, mice develop pancreatic intraepithelial neoplasia and microinvasive neoplasms

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
pancreatic ductal adenocarcinoma DOID:3498 J:186717




Genotype
MGI:7285921
cn3
Allelic
Composition
Usp9xtm1Tuv/Y
Tg(Ddx4-cre)1Dcas/0
Genetic
Background
involves: FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Ddx4-cre)1Dcas mutation (2 available)
Usp9xtm1Tuv mutation (0 available); any Usp9x mutation (24 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
• at 2 weeks of age or later, spermatogenic cells undergo apoptotic cell death at the early spermatocyte stage, as shown by TUNEL staining
• TUNEL+ spermatogenic cells are observed in the adluminal area nearest to the basal compartment throughout the postnatal to adult stages
• however, no differences in TUNEL+ cell number are observed in the newborn and 1-week-old stage
• at 12 weeks of age, ~85% of transverse sections of seminiferous tubules lack some spermatogenic cells
• ~3.3% of tubules contain both round and elongated spermatids but completely lack spermatocytes; ~68.8% of tubules contain a few spermatocytes and a few spermatids; and ~13.0% of tubules show almost complete lack of meiotic and post-meiotic spermatogenic cells in the adluminal compartment
• at 12 weeks of age, testes are grossly smaller than those in male controls
• at 12 weeks of age, testis weight x 100/body weight (%) is significantly lower than that in male controls
• testes show defective transition from the mitotic to meiotic phases and/or defective maintenance of early meiotic phase
• however, maintenance of undifferentiated GFRA1+/PLZF+ and differentiating KIT+ spermatogonia appear to be normal
• adult males contain only a few spermatozoa in the caudal epididymides
• some affected spermatids show aberrant condensed Hsc70t-positive signals as a residual body-like structure in the seminiferous tubules
• also, some elongated spermatids are degenerating and occasionally misaligned near the basement membrane, suggesting Sertoli cell phagocytosis of aberrant spermatids
• at 12 weeks of age, anti-SCP3 immunostaining showed that the relative number of spermatocytes is reduced to three-fourths of control testes
• however, spermatocytes that pass through meiotic differentiation and remain in affected seminiferous tubules show no defects in recombination and formation of the XY body
• PAS staining showed that some fully completed spermatozoa are abnormally retained in the seminiferous tubules containing the elongating spermatids at step 9-10, suggesting defects in spermiogenesis and/ or spermiation
• failure of spermiation subsequent to apoptotic cell death in early spermatocytes
• males are completely infertile
• however, vaginal plugs are observed in paired wild-type females

cellular
• adult males contain only a few spermatozoa in the caudal epididymides
• some affected spermatids show aberrant condensed Hsc70t-positive signals as a residual body-like structure in the seminiferous tubules
• also, some elongated spermatids are degenerating and occasionally misaligned near the basement membrane, suggesting Sertoli cell phagocytosis of aberrant spermatids
• at 12 weeks of age, anti-SCP3 immunostaining showed that the relative number of spermatocytes is reduced to three-fourths of control testes
• however, spermatocytes that pass through meiotic differentiation and remain in affected seminiferous tubules show no defects in recombination and formation of the XY body
• at 2 weeks of age or later, spermatogenic cells undergo apoptotic cell death at the early spermatocyte stage, as shown by TUNEL staining
• TUNEL+ spermatogenic cells are observed in the adluminal area nearest to the basal compartment throughout the postnatal to adult stages
• however, no differences in TUNEL+ cell number are observed in the newborn and 1-week-old stage

endocrine/exocrine glands
• at 12 weeks of age, ~85% of transverse sections of seminiferous tubules lack some spermatogenic cells
• ~3.3% of tubules contain both round and elongated spermatids but completely lack spermatocytes; ~68.8% of tubules contain a few spermatocytes and a few spermatids; and ~13.0% of tubules show almost complete lack of meiotic and post-meiotic spermatogenic cells in the adluminal compartment
• at 12 weeks of age, testes are grossly smaller than those in male controls
• at 12 weeks of age, testis weight x 100/body weight (%) is significantly lower than that in male controls





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last database update
04/30/2024
MGI 6.23
The Jackson Laboratory