All Phenotypes Poglut3<tm1.3Rsh> MGI Mouse

neonatal lethality ( J:350484 )

• newborns are viable but usually do not survive beyond P0

• however, E18.5 fetuses are viable and present at expected Mendelian ratios

decreased birth body size ( J:350484 )

• newborns are severely runted

decreased body size ( J:350484 )

• rare surviving mice are runted

decreased fetal size ( J:350484 )

• E18.5 fetuses are smaller

hypoactivity ( J:350484 )

• newborns are less active

syndactyly ( J:350484 )

• rare surviving mice have fully penetrant syndactyly between digits two and three in both the fore- and hindlimbs

abnormal eye morphology ( J:350484 )

• about 50% of E18.5 fetuses exhibit eye defects

abnormal lung morphology ( J:350484 )

• lung parenchyma from rare, surviving mice shows greatly reduced and fragmented elastic fibers with wide airspaces

• fibrillin is disorganized in E18.5 lung, lacking the characteristic scalloped organization in the bronchioles and appearing thinner in the saccule regions

abnormal lung vasculature morphology ( J:350484 )

• E18.5 lungs show abnormalities in the organization of cells in the blood vessels

• the pre-acinar blood vessel (pulmonary vein and pulmonary artery) walls are thicker at E18.5, with poorly arranged endothelium, loose and abnormal media, and aberrant internal and external layers compared to wild-type lung blood vessels

abnormal lung saccule morphology ( J:350484 )

• E18.5 lungs show abnormalities in the organization of cells in the saccules

• the saccule airspaces appear enlarged

• fibrillin appears thinner in the saccule regions at E18.5

abnormal pulmonary elastic fiber morphology ( J:350484 )

• 34% and 17% decrease in elastic fibers in the pulmonary blood vessels and in the pulmonary artery, respectively

• 38% and 39% decrease in elastic fibers in the bronchiole and saccules, respectively

abnormal bronchiole morphology ( J:350484 )

• E18.5 lungs show abnormalities in the organization of cells in the bronchioles

• the epithelial folds of bronchioles are flattened with reduced lamina propria at E18.5

• smooth muscle cells in the bronchiole appear round instead of elongated

• elastin deposition is more dispersed in the bronchiole than in wild-type bronchiole which shows intermittent elastic depositio

• fibrillin lacks the characteristic scalloped organization in the bronchioles at E18.5

abnormal blood vessel elastic tissue morphology ( J:350484 )

• E18.5 lungs show defects in the elastic fibers of the blood vessels and bronchiole, with sparse elastic laminae and extensive fragmentation in the internal elastic lamina and nearly punctate in the middle and external laminae

abnormal lung vasculature morphology ( J:350484 )

• E18.5 lungs show abnormalities in the organization of cells in the blood vessels

• the pre-acinar blood vessel (pulmonary vein and pulmonary artery) walls are thicker at E18.5, with poorly arranged endothelium, loose and abnormal media, and aberrant internal and external layers compared to wild-type lung blood vessels

abnormal vascular smooth muscle morphology ( J:350484 )

• smooth muscle cells in both the pulmonary artery appear round instead of elongated

abnormal vascular smooth muscle morphology ( J:350484 )

• smooth muscle cells in both the pulmonary artery appear round instead of elongated

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

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

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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