Mouse Genome Informatics
involves: 129S2/SvPas
phenotype observed in females
phenotype observed in males
N normal phenotype

Growth retardation in Fut8tm1Nta/Fut8tm1Nta mice

• about 70% die within 3 days of age

• most homozygotes surviving past 3 days of age manifest severe growth retardation

respiratory system
• exhibit dilated alveolar ducts
• increase in the diameters of pulmonary alveoli from P7
• exhibit progressive emphysema-like changes, including impaired respiration and enhanced expression of matrix metalloproteases
• exhibit fragmentation and reduction in the number of elastic fibers in the lungs
• exhibit generalized air-space enlargement in the lungs
• lungs have larger total lung capacities
• display an increase in lung compliance
• ventilatory responses to systemic hypoxia or hypercapnia, or increases in the respiratory minute volume, are significantly attenuated
• under resting conditions, respiratory minute volume and rate are higher than in wild-type

Mouse Models of Human Disease
Emphysema, Hereditary Pulmonary 130700 J:102932