All Phenotypes Flnb<Gt(BGB085)Byg> MGI Mouse

premature death ( J:120066 )

• mice that survived past birth, do not survive beyond 4 weeks of age

perinatal lethality, incomplete penetrance ( J:120066 )

• less than 3% of mice reach term

embryonic lethality during organogenesis, incomplete penetrance ( J:120066 )

• at E11.5 there is a decrease in the percentage of homozygous embryos

decreased body weight ( J:120066 )

• weight is reduced by 2- to 3-fold compared to heterozygotes and wild-type mice at 4 weeks of age

short tibia ( J:120066 )

• 4-week-old mice have reduced tibia

• mid-diaphyseal area has significantly reduced bone mineral density with reduced cortical thickness

• however, there is no change in the trabecular volume

abnormal vertebral column morphology ( J:120066 )

• skeletal malformation in the vertebral column including kyphotic changes in the thoracic part and scoliotic changes in the abdominal part

absent intervertebral disk ( J:120066 )

• at E13.5 intervertebral discs, including the nuclei pulposus and surrounding cartilage, are absent

• postnatal mice lack intervertebral disks in scoliotic and kyphotic areas of the spine

kyphoscoliosis ( J:120066 )

• kyphotic changes in the thoracic part and scoliotic changes in the abdominal part of the vertebral column

vertebral fusion ( J:120066 )

• resulting from absence of the intervertebral disks

decreased bone mineral density ( J:120066 )

• mid-diaphyseal area of the tibia has significantly reduced bone mineral density

decreased compact bone thickness ( J:120066 )

• in the mid-diaphyseal area of the tibia at 4 weeks of age

• however, there is no change in the trabecular bone volume of the tibia

abnormal hyaline cartilage morphology ( J:120066 )

• hyaline cartilage is reduced in ribs, metacarpal bones, phalanges, and tarsal bones

abnormal brain vasculature morphology ( J:120066 )

• at E11.5 microvascular networks in the central nervous system are poorly developed

abnormal middle cerebral artery morphology ( J:120066 )

• at E13.5 the middle cerebral artery is enlarged and rudimentary

abnormal angiogenesis ( J:120066 )

• at E11.5 the microvascular networks in the central nervous system and perivertebral areas are poorly developed

• at E11.5, sprouting of the microvessels is severely impaired

• at E13.5 the microvascular networks in the perivertebral area are disorganized and poorly developed

abnormal artery development ( J:120066 )

• at E13.5, development of the larger blood vessels, such as the middle cerebral artery, is impaired

abnormal motor capabilities/coordination/movement ( J:120066 )

• limited movement

abnormal posture ( J:120066 )

• rigid posture due to fusion of vertebral bodies

abnormal cell physiology ( J:120066 )

• serum-induced stress fibers in fibroblasts show greater disorganization in the formation of filamentous actin compared to wild-type cells

decreased fibroblast cell migration ( J:120066 )

• 75% reduction in fibroblast migration as judged by a Boyden chamber assay and a 20% reduction in migration of fibroblasts in a wound-healing assay