Phenotypes Associated with This Genotype

Genotype
MGI:3583005

• Allelic Composition: Mmp14^tm1Hbh/Mmp14^tm1Hbh
• Genetic Background: involves: 129P1/ReJ * 129P2/OlaHsd * Black Swiss

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

respiratory system

impaired lung alveolus development ( J:97067 )

• demonstrated only a modest increase in alveolization from P1 to P14, however proximal lung branching was normal

abnormal club cell morphology ( J:97067 )

• Clara cells were flattened and lacked apical protrusions at the level of the terminal bronchiole

pulmonary hypoplasia ( J:97067 )

dilated pulmonary alveolar duct ( J:97067 )

• enlarged alveolar ducts

abnormal pulmonary alveolus morphology ( J:97067 )

• 40% less alveolar surface area and 46% greater alveolar diameter at 1 month of age with these differences persisting in adulthood and the surface area declining even more over time

overexpanded pulmonary alveolus ( J:97067 )

• 46% greater alveolar diameter at 1 month of age with difference persisting in adulthood

absent alveolar pores ( J:97067 )

• failed to develop pores of Kohn

abnormal pulmonary elastic fiber morphology ( J:97067 )

• showed slightly increased thickness of elastic fibers in alveolar walls

abnormal terminal bronchiole morphology ( J:97067 )

• Clara cells were flattened and lacked apical protrusions at the level of the terminal bronchiole

dilated respiratory conducting tube ( J:97067 )

• enlarged distal airways and the terminal airways extended to the pleural surface