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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/Rb1tm2Brn
Trp53tm1Brn/Trp53tm1Brn
Tg(Col1a1-cre)1Kry/0 		involves: 129 * 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796167
cn3
 		Prkar1atm1.2Lsk/Prkar1a+
Rb1tm2Brn/Rb1+
Tg(Col1a1-cre)1Kry/0
Trp53tm1Brn/Trp53tm1Brn 		involves: 129 * 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796166
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/Prkar1atm1.2Lsk
Tg(Ghrhr-cre)3242Lsk/0 		involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3771850
cn6
 		Prkar1atm1.2Lsk/Prkar1a+
Tg(Bglap2-TAg)1Rkho/0
Tg(Col1a1-cre)1Kry/0 		involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796026
cn7
 		Prkar1atm1.2Lsk/Prkar1a+
Tg(Col1a1-cre)1Kry/0 		involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796038
cn8
 		Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(Col1a1-cre)1Kry/0 		involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:5796037
cn9
 		Prkar1atm1.2Lsk/Prkar1a+
Ptentm1.1Mwst/Pten+
Tg(TPO-cre)1Shk/0 		involves: 129S1/Sv * 129X1/SvJ * FVB/NCr MGI:5897775
cn10
 		Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Tg(TPO-cre)1Shk/0 		involves: 129S1/Sv * 129X1/SvJ * FVB/NCr MGI:5897776
cn11
 		Prkar1atm1.2Lsk/Prkar1atm1.2Lsk
Ptentm1.1Mwst/Ptentm1.1Mwst
Tg(TPO-cre)1Shk/0 		involves: 129S1/Sv * 129X1/SvJ * FVB/NCr MGI:5897675


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 (1 available); any Prkar1a mutation (19 available)
Tg(Tyr-cre)3Gfk mutation (0 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
neoplasm
increased facial tumor incidence ( J:98799 )
• unilateral or bilateral tumors on the side of the face starting at 3 - 4 months
increased Schwannoma incidence ( J:98799 )
• tumors in subcutaneous tissue - Schwannomas

nervous system
increased Schwannoma incidence ( J:98799 )
• tumors in subcutaneous tissue - Schwannomas

growth/size/body
increased facial tumor incidence ( J:98799 )
• unilateral or bilateral tumors on the side of the face starting at 3 - 4 months

craniofacial
increased facial tumor incidence ( J:98799 )
• unilateral or bilateral tumors on the side of the face starting at 3 - 4 months

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:5796167
cn2
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 (1 available); any Prkar1a mutation (19 available)
Rb1tm2Brn mutation (3 available); any Rb1 mutation (111 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
Trp53tm1Brn mutation (18 available); any Trp53 mutation (240 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
premature death ( J:234128 )
• mice do not live longer than 11 weeks
• treatment of mice with RANK-Fc prolongs survival to around to around 13.3 weeks

neoplasm
increased osteosarcoma incidence ( J:234128 )
• 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
increased osteosarcoma incidence ( J:234128 )
• 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:5796166
cn3
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 (1 available); any Prkar1a mutation (19 available)
Rb1tm2Brn mutation (3 available); any Rb1 mutation (111 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
Trp53tm1Brn mutation (18 available); any Trp53 mutation (240 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
premature death ( J:234128 )
• mice survive to around 13.9 weeks
• treatment of mice with RANK-Fc prolongs survival to around 17.8 weeks

neoplasm
increased osteosarcoma incidence ( J:234128 )
• 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
increased osteosarcoma incidence ( J:234128 )
• 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
increased cell migration ( J:234128 )
• 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: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 (1 available); any Prkar1a mutation (19 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
Trp53tm1Brn mutation (18 available); any Trp53 mutation (240 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
premature death ( J:234128 )
• mice survive to around 17 weeks of age

neoplasm
increased osteosarcoma incidence ( J:234128 )
• 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
increased osteosarcoma incidence ( J:234128 )
• 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:3771850
cn5
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 (1 available); any Prkar1a mutation (19 available)
Tg(Ghrhr-cre)3242Lsk mutation (1 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
neoplasm
increased pituitary gland tumor incidence ( J:130264 )
• however, the number of prolactinomas is not increased relative to in wild-type mice
• when pituitary glands are examined, mice exhibit an increased rate of tumorigenesis (in 48% of mice compared to 30.6% of wild-type mice)
• nonprolactonmas stain positive for multiple hormone subtypes including Prl, GH and TSH

homeostasis/metabolism
increased circulating growth hormone level ( J:130264 )
• 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)

endocrine/exocrine glands
increased pituitary gland tumor incidence ( J:130264 )
• 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
increased pituitary gland tumor incidence ( J:130264 )
• 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




Genotype
MGI:5796026
cn6
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 (1 available); any Prkar1a mutation (19 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
premature death ( J:165282 )
• no mice survive past 5 weeks of age

neoplasm
increased osteosarcoma incidence ( J:165282 )
• 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
increased osteosarcoma incidence ( J:165282 )
• 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
abnormal motor capabilities/coordination/movement ( J:165282 )
• impaired mobility due to osteosarcoma in the spine
paralysis ( J:165282 )
• paralysis due to osteosarcoma in the spine




Genotype
MGI:5796038
cn7
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 (1 available); any Prkar1a mutation (19 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
increased skeletal tumor incidence ( J:165282 )
• 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
increased skeletal tumor incidence ( J:165282 )
• 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
abnormal bone remodeling ( J:165282 )
• bone within and around lesions is normal and contains osteocytes but is undergoing extensive remodeling




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 (1 available); any Prkar1a mutation (19 available)
Tg(Col1a1-cre)1Kry mutation (2 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
neonatal lethality, complete penetrance ( J:165282 )
• mice die within 24 hours of birth




Genotype
MGI:5897775
cn9
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 (1 available); any Prkar1a mutation (19 available)
Ptentm1.1Mwst mutation (0 available); any Pten mutation (88 available)
Tg(TPO-cre)1Shk mutation (1 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
decreased survivor rate ( J:225245 )
• mice exhibit reduced survival compared to single heterozygotes

neoplasm
neoplasm ( J:225245 )
N
• mice do not show increased incidence of thyroid cancer




Genotype
MGI:5897776
cn10
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 (1 available); any Prkar1a mutation (19 available)
Tg(TPO-cre)1Shk mutation (1 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
endocrine/exocrine glands
abnormal thyroid gland morphology ( J:241066 )
• thyroids show increased cellularity but retain a follicular pattern of growth
enlarged thyroid gland ( J:241066 )
• 100% of mice exhibit enlargement of the thyroid
increased thyroid carcinoma incidence ( J:225245 , J:241066 )
• 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)
increased activity of thyroid gland ( J:241066 )
• mice are hyperthyroid

homeostasis/metabolism
increased thyroxine level ( J:241066 )
• T4 levels are elevated, indicating hyperthyroidism
decreased thyroid-stimulating hormone level ( J:241066 )
• TSH levels are lower but not statistically significant due to wide variation in wild-type mice

neoplasm
increased thyroid carcinoma incidence ( J:225245 , J:241066 )
• 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




Genotype
MGI:5897675
cn11
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 (1 available); any Prkar1a mutation (19 available)
Ptentm1.1Mwst mutation (0 available); any Pten mutation (88 available)
Tg(TPO-cre)1Shk mutation (1 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
premature death ( J:225245 )
• median survival age of 6 months

growth/size/body
weight loss ( J:225245 )
• males exhibit lower body weight beginning at 1 month of age which becomes statistically significant at 5 months of age

endocrine/exocrine glands
increased thyroid carcinoma incidence ( J:225245 )
• 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
increased activity of thyroid gland ( J:225245 )
• mice are hyperthyroid

adipose tissue
decreased subcutaneous adipose tissue amount ( J:225245 )
• reduction in subcutaneous adipose tissue
decreased abdominal adipose tissue amount ( J:225245 )
• reduction in visceral adipose tissue

homeostasis/metabolism
increased circulating thyroxine level ( J:225245 )
• free T4 levels are increased

integument
decreased subcutaneous adipose tissue amount ( J:225245 )
• reduction in subcutaneous adipose tissue

neoplasm
increased thyroid carcinoma incidence ( J:225245 )
• 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
increased metastatic potential ( J:225245 )
• 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




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Send questions and comments to User Support. 		last database update
11/12/2024
MGI 6.24 		The Jackson Laboratory