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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Prkar1atm1.2Lsk
targeted mutation 1.2, Lawrence Kirschner
MGI:3512112
Summary 11 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(Tyr-cre)3Gfk/0
either: (involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA) or (involves: 129S1/Sv * 129X1/SvJ * C57BL/6) MGI:3580534
cn2
Prkar1atm1.2Lsk/Prkar1a+
Rb1tm2Brn/Rb1+
Tg(Col1a1-cre)1Kry/0
Trp53tm1Brn/Trp53tm1Brn
involves: 129 * 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796166
cn3
Prkar1atm1.2Lsk/Prkar1a+
Rb1tm2Brn/Rb1tm2Brn
Trp53tm1Brn/Trp53tm1Brn
Tg(Col1a1-cre)1Kry/0
involves: 129 * 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796167
cn4
Prkar1atm1.2Lsk/Prkar1a+
Tg(Col1a1-cre)1Kry/0
Trp53tm1Brn/Trp53tm1Brn
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796169
cn5
Prkar1atm1.2Lsk/Prkar1a+
Tg(Col1a1-cre)1Kry/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796038
cn6
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(Ghrhr-cre)3242Lsk/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3771850
cn7
Prkar1atm1.2Lsk/Prkar1a+
Tg(Bglap2-TAg)1Rkho/0
Tg(Col1a1-cre)1Kry/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796026
cn8
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(Col1a1-cre)1Kry/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796037
cn9
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Ptentm1.1Mwst/Ptentm1.1Mwst
Tg(TPO-cre)1Shk/0
involves: 129S1/Sv * 129X1/SvJ * FVB/NCr MGI:5897675
cn10
Prkar1atm1.2Lsk/Prkar1a+
Ptentm1.1Mwst/Pten+
Tg(TPO-cre)1Shk/0
involves: 129S1/Sv * 129X1/SvJ * FVB/NCr MGI:5897775
cn11
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(TPO-cre)1Shk/0
involves: 129S1/Sv * 129X1/SvJ * FVB/NCr MGI:5897776


Genotype
MGI:3580534
cn1
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(Tyr-cre)3Gfk/0
Genetic
Background
either: (involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA) or (involves: 129S1/Sv * 129X1/SvJ * C57BL/6)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Tg(Tyr-cre)3Gfk mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
craniofacial
• unilateral or bilateral tumors on the side of the face starting at 3 - 4 months

nervous system
• tumors in subcutaneous tissue - Schwannomas

growth/size/body
• unilateral or bilateral tumors on the side of the face starting at 3 - 4 months

neoplasm
• unilateral or bilateral tumors on the side of the face starting at 3 - 4 months
• tumors in subcutaneous tissue - Schwannomas

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Carney complex DOID:0050471 OMIM:160980
OMIM:605244
OMIM:608837
J:98799




Genotype
MGI:5796166
cn2
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1a+
Rb1tm2Brn/Rb1+
Tg(Col1a1-cre)1Kry/0
Trp53tm1Brn/Trp53tm1Brn
Genetic
Background
involves: 129 * 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Rb1tm2Brn mutation (3 available); any Rb1 mutation (65 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
Trp53tm1Brn mutation (7 available); any Trp53 mutation (153 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice survive to around 13.9 weeks
• treatment of mice with RANK-Fc prolongs survival to around 17.8 weeks

neoplasm
• mice develop osteosarcoma within 10.3 weeks of age
• tumors are marked by high TNFSF11 (RANKL)/low TNFRSF11A (RANK) levels
• treatment of mice with RANK-Fc for 52 weeks beginning at 12.6 weeks of age keeps mice tumor-free until 64.6 weeks of age

skeleton
• mice develop osteosarcoma within 10.3 weeks of age
• tumors are marked by high TNFSF11 (RANKL)/low TNFRSF11A (RANK) levels
• treatment of mice with RANK-Fc for 52 weeks beginning at 12.6 weeks of age keeps mice tumor-free until 64.6 weeks of age

cellular
• in a scratch wound healing assay, tumor cells show greater migration than tumor cells from conditional Rb1tm2Brn heterozygous Trp53tm1Brn homozygous double mutants

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
osteosarcoma DOID:3347 OMIM:259500
J:234128




Genotype
MGI:5796167
cn3
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1a+
Rb1tm2Brn/Rb1tm2Brn
Trp53tm1Brn/Trp53tm1Brn
Tg(Col1a1-cre)1Kry/0
Genetic
Background
involves: 129 * 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Rb1tm2Brn mutation (3 available); any Rb1 mutation (65 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
Trp53tm1Brn mutation (7 available); any Trp53 mutation (153 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice do not live longer than 11 weeks
• treatment of mice with RANK-Fc prolongs survival to around to around 13.3 weeks

neoplasm
• mice develop osteosarcoma within 6.3 weeks of age
• tumors are marked by high (TNFSF11) RANKL/low (TNFRSF11A) RANK levels
• treatment of mice with RANK-Fc starting at 2.4 weeks of age suppresses tumor growth
• mice develop tumors 4.3 weeks after the discontinuation of RANK-Fc treatment and large numbers of osteoclasts are seen lining the boundary of tumor cells and adjacent osteopetrotic bone

skeleton
• mice develop osteosarcoma within 6.3 weeks of age
• tumors are marked by high (TNFSF11) RANKL/low (TNFRSF11A) RANK levels
• treatment of mice with RANK-Fc starting at 2.4 weeks of age suppresses tumor growth
• mice develop tumors 4.3 weeks after the discontinuation of RANK-Fc treatment and large numbers of osteoclasts are seen lining the boundary of tumor cells and adjacent osteopetrotic bone

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
osteosarcoma DOID:3347 OMIM:259500
J:234128




Genotype
MGI:5796169
cn4
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1a+
Tg(Col1a1-cre)1Kry/0
Trp53tm1Brn/Trp53tm1Brn
Genetic
Background
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
Trp53tm1Brn mutation (7 available); any Trp53 mutation (153 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice survive to around 17 weeks of age

neoplasm
• mice develop osteosarcoma within 17 weeks of age
• multiple tumors among long bones, spine, jaw, and skull
• tumors are composed of malignant-appearing osteoblasts with woven bone as the predominant matrix and occasional chondroblastic or fibroblastic phenotype
• tumors are marked by high TNFSF11 (RANKL)/low TNFRSF11A (RANK) levels

skeleton
• mice develop osteosarcoma within 17 weeks of age
• multiple tumors among long bones, spine, jaw, and skull
• tumors are composed of malignant-appearing osteoblasts with woven bone as the predominant matrix and occasional chondroblastic or fibroblastic phenotype
• tumors are marked by high TNFSF11 (RANKL)/low TNFRSF11A (RANK) levels

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
osteosarcoma DOID:3347 OMIM:259500
J:234128




Genotype
MGI:5796038
cn5
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1a+
Tg(Col1a1-cre)1Kry/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Bone tumorigenesis nd abundant osteoclasts in Prkar1atm1.2Lsk/Prkar1a+ Tg(Col1a1-cre)1Kry/0 mice

neoplasm
• mice develop bone tumors starting at 10 weeks of age, with most mice having tumors by 40 weeks of age
• lesions grow slowly, with 25% of mice having 8-mm tumors by 50 weeks of age
• mice do not develop metastases
• lesions are not typical osteosarcomas as they are not composed of malignant osteoblasts depositing osteoid, but consist of an enlarged marrow space filled with abundant myxoid matrix, containing small fibroblast-like cells surrounded by trabecular-like bone, lined by normal osteoblasts and regions with abundant osteoclasts

skeleton
• mice develop bone tumors starting at 10 weeks of age, with most mice having tumors by 40 weeks of age
• lesions grow slowly, with 25% of mice having 8-mm tumors by 50 weeks of age
• mice do not develop metastases
• lesions are not typical osteosarcomas as they are not composed of malignant osteoblasts depositing osteoid, but consist of an enlarged marrow space filled with abundant myxoid matrix, containing small fibroblast-like cells surrounded by trabecular-like bone, lined by normal osteoblasts and regions with abundant osteoclasts
• bone within and around lesions is normal and contains osteocytes but is undergoing extensive remodeling




Genotype
MGI:3771850
cn6
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(Ghrhr-cre)3242Lsk/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Tg(Ghrhr-cre)3242Lsk mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
endocrine/exocrine glands
• when pituitary glands are examined, mice exhibit an increased rate of tumorigenesis (in 48% of mice compared to 30.6% of wild-type mice)
• however, the number of prolactinomas is not increased relative to in wild-type mice
• nonprolactonmas stain positive for multiple hormone subtypes including Prl, GH and TSH

nervous system
• when pituitary glands are examined, mice exhibit an increased rate of tumorigenesis (in 48% of mice compared to 30.6% of wild-type mice)
• however, the number of prolactinomas is not increased relative to in wild-type mice
• nonprolactonmas stain positive for multiple hormone subtypes including Prl, GH and TSH

neoplasm
• when pituitary glands are examined, mice exhibit an increased rate of tumorigenesis (in 48% of mice compared to 30.6% of wild-type mice)
• however, the number of prolactinomas is not increased relative to in wild-type mice
• nonprolactonmas stain positive for multiple hormone subtypes including Prl, GH and TSH

homeostasis/metabolism
• regardless of tumor status mice exhibit increased serum growth hormone levels (28.27+/-41.4 ng/ml compared to 8.44+/-20.7 ng/ml in wild-type mice)




Genotype
MGI:5796026
cn7
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1a+
Tg(Bglap2-TAg)1Rkho/0
Tg(Col1a1-cre)1Kry/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Tg(Bglap2-TAg)1Rkho mutation (0 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Prkar1a heterozygosity accelerates osteosarcoma development in Tg(Bglap2-TAg)1Rkho/0 mice

mortality/aging
• no mice survive past 5 weeks of age

neoplasm
• mice develop advanced osteosarcoma, often in the spine
• mice exhibit multi-ostotic tumors in long and flat bones, including the skull, vertebrae, ribs, and tibia
• however, metastasis is not seen at this early age
• tumors show increased osteoclast numbers

skeleton
• mice develop advanced osteosarcoma, often in the spine
• mice exhibit multi-ostotic tumors in long and flat bones, including the skull, vertebrae, ribs, and tibia
• however, metastasis is not seen at this early age
• tumors show increased osteoclast numbers

behavior/neurological
• impaired mobility due to osteosarcoma in the spine
• paralysis due to osteosarcoma in the spine




Genotype
MGI:5796037
cn8
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(Col1a1-cre)1Kry/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die within 24 hours of birth




Genotype
MGI:5897675
cn9
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Ptentm1.1Mwst/Ptentm1.1Mwst
Tg(TPO-cre)1Shk/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/NCr
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Ptentm1.1Mwst mutation (0 available); any Pten mutation (49 available)
Tg(TPO-cre)1Shk mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• median survival age of 6 months

growth/size/body
• males exhibit lower body weight beginning at 1 month of age which becomes statistically significant at 5 months of age

endocrine/exocrine glands
• 100% of mice develop follicular thyroid carcinoma by 8 weeks of age, showing capsular and/or vascular invasion
• tumors show a molecular signature similar to human sporadic follicular thyroid cancer
• mice are hyperthyroid

adipose tissue
• reduction in visceral adipose tissue
• reduction in subcutaneous adipose tissue

homeostasis/metabolism
• free T4 levels are increased

integument
• reduction in subcutaneous adipose tissue

neoplasm
• 100% of mice develop follicular thyroid carcinoma by 8 weeks of age, showing capsular and/or vascular invasion
• tumors show a molecular signature similar to human sporadic follicular thyroid cancer
• metastatic follicular thyroid carcinoma is seen in the lungs of 27% of mice; metastases are well differentiated, maintain follicular structure with colloid, and stain for thyroglobulin
• tumors exhibit only mild increases in proliferation rate compared to those in single homozygous Prkar1atm1.2Lsk Tg(TPO-cre)1Shk/0 mice

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
follicular thyroid carcinoma DOID:3962 OMIM:188470
J:241066




Genotype
MGI:5897775
cn10
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1a+
Ptentm1.1Mwst/Pten+
Tg(TPO-cre)1Shk/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/NCr
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Ptentm1.1Mwst mutation (0 available); any Pten mutation (49 available)
Tg(TPO-cre)1Shk mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice exhibit reduced survival compared to single heterozygotes

neoplasm
N
• mice do not show increased incidence of thyroid cancer




Genotype
MGI:5897776
cn11
Allelic
Composition
Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(TPO-cre)1Shk/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/NCr
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1.2Lsk mutation (0 available); any Prkar1a mutation (10 available)
Tg(TPO-cre)1Shk mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
endocrine/exocrine glands
• thyroids show increased cellularity but retain a follicular pattern of growth
• 100% of mice exhibit enlargement of the thyroid
• thyroids exhibit locally invasive follicular thyroid carcinoma (J:225245)
• tumors exhibit high proliferation rate (J:225245)
• mice develop follicular thyroid carcinoma at a rate of 43% by one year of age (J:241066)
• tumors exhibit increased proliferation compared to wild-type thyroid glands (J:241066)
• however, none of the tumors show widely invasive or angio-invasive behavior (J:241066)
• mice are hyperthyroid

homeostasis/metabolism
• TSH levels are lower but not statistically significant due to wide variation in wild-type mice
• T4 levels are elevated, indicating hyperthyroidism

neoplasm
• thyroids exhibit locally invasive follicular thyroid carcinoma (J:225245)
• tumors exhibit high proliferation rate (J:225245)
• mice develop follicular thyroid carcinoma at a rate of 43% by one year of age (J:241066)
• tumors exhibit increased proliferation compared to wild-type thyroid glands (J:241066)
• however, none of the tumors show widely invasive or angio-invasive behavior (J:241066)

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
follicular thyroid carcinoma DOID:3962 OMIM:188470
J:241066





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last database update
08/15/2017
MGI 6.10
The Jackson Laboratory