Phenotypes associated with this allele

• Allele Symbol: Fstl1^tm1.1Ning
• Allele Name: targeted mutation 1.1, Wen Ning
• Allele ID: MGI:4950852
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Fstl1^tm1.1Ning/Fstl1^tm1.1Ning	involves: 129S4/SvJae * C57BL/6J * FVB/N	MGI:4950867

Genotype
MGI:4950867

hm1

• Allelic Composition: Fstl1^tm1.1Ning/Fstl1^tm1.1Ning
• Genetic Background: involves: 129S4/SvJae * C57BL/6J * FVB/N

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

Fstl1^tm1.1Ning/Fstl1^tm1.1Ning neonates are cyanotic

mortality/aging

neonatal lethality, complete penetrance ( J:171347 )

respiratory system

abnormal larynx morphology ( J:171347 )

• disorganized

abnormal lung morphology ( J:171347 )

• at E18.5, air sac spaces are reduced 60% and intersaccular septa is thickened and hypercellular compared to in wild-type mice

• from saccular stage (E17.5 to birth), lungs start to exhibit condensation unlike in wild-type mice

pulmonary hyperplasia ( J:171347 )

• lungs are hypercellular due to epithelial cell hyperplasia

abnormal lung development ( J:171347 )

• distal epithelial differentiation in the lungs is impaired compared to in wild-type mice

• maturation of type II alveolar cells is delayed and differentiation of type I alveolar cells is reduced compared to in wild-type mice

abnormal lung epithelium morphology ( J:171347 )

• at E15.5 and E18.5, the number of epithelial cells in the lungs is increased due to increased proliferation compared to in wild-type mice

decreased type I pneumocyte number ( J:171347 )

abnormal type II pneumocyte morphology ( J:171347 )

• type II alveolar cells are immature with smaller apical microvilli, fewer developing lamellar bodies, and dispersed cytoplasmic glycogen compared to in wild-type mice

decreased alveolar lamellar body number ( J:171347 )

increased type II pneumocyte number ( J:171347 )

abnormal pulmonary alveolar sac morphology ( J:171347 )

• at E18.5, air sac spaces are reduced 60% compared to in wild-type mice

primary atelectasis ( J:171347 )

• however, reducing BMP signaling activity rescues pulmonary atelectasis

abnormal bronchiole morphology ( J:171347 )

• overexpanded

abnormal trachea morphology ( J:171347 )

• enlarged as early as E15.5

• deformed and enlarged lumen by E18.5

• tracheal tubes are soft and flabby unlike in wild-type mice

• the upper and lower ends of the trachea is enlarged while the middle second is narrow with irregular inner margins

abnormal tracheal ciliated epithelium morphology ( J:171347 )

• at E18.5, mice exhibit disorganized tracheal cartilage epithelium with shorter and disordered cilia compared with wild-type mice

abnormal tracheal cartilage morphology ( J:171347 )

• interrupted or truncated at E15.5 and E18.5

• cartilage rings do not grow and extend dorsally unlike in wild-type mice

decreased tracheal cartilage ring number ( J:171347 )

trachea stenosis ( J:171347 )

• at birth

abnormal breathing pattern ( J:171347 )

• mice exhibit irregular breathing

decreased surfactant secretion ( J:171347 )

• at E18.5

skeleton

abnormal skeleton morphology ( J:171347 )

abnormal tracheal cartilage morphology ( J:171347 )

• interrupted or truncated at E15.5 and E18.5

• cartilage rings do not grow and extend dorsally unlike in wild-type mice

decreased tracheal cartilage ring number ( J:171347 )

homeostasis/metabolism

cyanosis ( J:171347 )

increased glycogen level ( J:171347 )

• immature type II alveolar cells exhibit increased glycogen content compared with wild-type cells

nervous system

abnormal neural tube morphology ( J:171347 )

• dorsal-ventral patterning is abnormal compared with wild-type mice

renal/urinary system

hydroureter ( J:171347 )

embryo

abnormal neural tube morphology ( J:171347 )

• dorsal-ventral patterning is abnormal compared with wild-type mice

growth/size/body

pulmonary hyperplasia ( J:171347 )

• lungs are hypercellular due to epithelial cell hyperplasia