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Phenotypes Associated with This Genotype
involves: 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkar1atm1Gsm mutation (1 available); any Prkar1a mutation (9 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Tumors in Prkar1atm1Gsm/Prkar1a+ mice

cardiovascular system
• depressed heart rate variability, however exhibit no heart rate changes, conduction delay or any cardiac myxomas

reproductive system

• increase in frequency of extracardiac tumors; 12 of 17 mutants developed tumors
• 5 of 12 mutants that develop tumors have hepatocellular carcinomas
• 3 of 12 mutants that develop tumors have soft tissue sarcomas with myxomatous differentiation invading the calvaria
• 2 of 12 mutants that develop tumors have paraspinal and spinal sarcomas (chondro- and meningeal saracomas) with cord compression and lower limb paralysis
• 2 of 12 mutants that develop tumors have s.c. soft tissue myxoid fibrosarcomas of the forelimb
• 4 of 12 mutants that develop tumors have hemangiosarcomas of the spleen with myxoid regions
• splenic hemangiosarcomas are associated with metachronous lesions including colonic, ovarian, and pulmonary hemangiosarcomas

• pigmentation is normal

Mouse Models of Human Disease
OMIM ID Ref(s)
Carney Complex, Type 1; CNC1 160980 J:93393

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.01
The Jackson Laboratory