Phenotypes Associated with This Genotype

Genotype
MGI:5502424

• Allelic Composition: Pkd1^tm2Ggg/Pkd1⁺; Tg(Col1a1-cre)1Bek/0
• Genetic Background: involves: 129S4/SvJae * CD-1

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

Decrease in trabecular and cortical bone in adult Pkd1^tm2Ggg/Pkd1⁺ Tg(Col1a1-cre)1Bek/0 and Pkd1^tm2Ggg/Pkd1^tm2Ggg Tg(Col1a1-cre)1Bek/0 mice

skeleton

impaired osteoblast differentiation ( J:191967 )

• osteoblasts show impaired differentiation and maturation, as shown by culture duration-dependent reductions in ALP activity, diminished calcium deposition in extracellular matrix and a reduction in osteoblastic differentiation markers

increased osteoblast proliferation ( J:191967 )

• primary calvarial osteoblasts exhibit an increase in BrdU incorporation

decreased bone mineral density ( J:191967 )

• bone mineral density is reduced by 21-22% in both males and females at 6 weeks of age

decreased trabecular bone volume ( J:191967 )

♂ • males show a 40% reduction in trabecular bone volume

decreased compact bone thickness ( J:191967 )

♂ • males show a 15% reduction in cortical bone thickness

decreased bone mineralization ( J:191967 )

• periosteal mineral apposition rate is reduced by 19%

cellular

impaired osteoblast differentiation ( J:191967 )

• osteoblasts show impaired differentiation and maturation, as shown by culture duration-dependent reductions in ALP activity, diminished calcium deposition in extracellular matrix and a reduction in osteoblastic differentiation markers

increased osteoblast proliferation ( J:191967 )

• primary calvarial osteoblasts exhibit an increase in BrdU incorporation

endocrine/exocrine glands

endocrine/exocrine gland phenotype ( J:191967 )

N • pancreatic cysts are not observed in embryos or at 6 weeks of age

renal/urinary system

renal/urinary system phenotype ( J:191967 )

N • kidney cysts are not observed in embryos or at 6 weeks of age