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Phenotypes Associated with This Genotype
Genotype
MGI:6404118
Allelic
Composition
Lrp4tm1.1Pg/Lrp4tm1.1Pg
Genetic
Background
involves: C57BL/6N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lrp4tm1.1Pg mutation (1 available); any Lrp4 mutation (60 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
skeleton
N
• no major skeletal abnormalities are seen in males at 5 months of age, with no bone syndactyly
• skull shows increased calvarial thickness
• femurs show increase in cortical thickness
• however, femurs show no difference in cortical porosity or in cortical bone mineral density
• increase in femoral midshaft diameter
• femurs and vertebral bodies show an increase in trabecular bone volume/tissue volume
• only 0.9% of osteocytes are sclerostin-positive compared to 30.1% of osteocytes in the trabeculae of the tibia of wild-type mice
• vertebral bodies show an increase in trabecular number
• vertebral bodies show a decrease in trabecular separation
• femurs show an increase in trabecular connectivity density
• femurs and vertebral bodies show an increase in trabecular thickness
• however, no difference in trabecular bone mineral density, trabecular number or separation of the femur is seen
• vertebral bodies show an increase in bone formation rate, composed of increased mineral apposition rate
• vertebral bodies show an increase in the osteoblast surface per bone surface, indicating an increase in bone formation by osteoblasts
• however, no significant changes in bone formation and resorption makers in the serum
• the force and the work or energy needed to fracture bones and the elastic modulus in a three-point bending analysis are increased, indicating increased bone strength

craniofacial
• skull shows increased calvarial thickness

homeostasis/metabolism
• mice show increased levels of sclerostin in the blood

limbs/digits/tail
• femurs show increase in cortical thickness
• however, femurs show no difference in cortical porosity or in cortical bone mineral density
• increase in femoral midshaft diameter

muscle
N
• mice show no signs of facial distortion or muscle paralysis

renal/urinary system
N
• kidneys appear normal and serum creatinine levels are normal, indicating normal renal function

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
sclerosteosis 2 DOID:0060757 OMIM:614305
J:277372


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
05/10/2022
MGI 6.19
The Jackson Laboratory