Phenotypes associated with this allele

• Allele Symbol: Tg(FABPCFTR)1Jaw
• Allele Name: transgene insertion 1, Jeffrey A Whitsett
• Allele ID: MGI:3052981
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Cftr^tm1Unc/Cftr^tm1Unc Tg(FABPCFTR)1Jaw/0 Tg(Scgb1a1-Scnn1b)6608Bouc/0	involves: 129P2/OlaHsd * C3H * C57BL/6 * FVB/N	MGI:5698394
cx2	Cftr^tm1Unc/Cftr^tm1Unc Tg(FABPCFTR)1Jaw/0	involves: 129P2/OlaHsd * C57BL/6 * FVB/N	MGI:3801014

Genotype
MGI:5698394

cx1

• Allelic Composition: Cftr^tm1Unc/Cftr^tm1Unc; Tg(FABPCFTR)1Jaw/0; Tg(Scgb1a1-Scnn1b)6608Bouc/0
• Genetic Background: involves: 129P2/OlaHsd * C3H * C57BL/6 * FVB/N

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

mortality/aging

postnatal lethality, incomplete penetrance ( J:191673 )

• survival is reduced by about 70% at P3

respiratory system

pulmonary epithelial necrosis ( J:191673 )

• airway necrosis is increased compared to single Tg(Scgb1a1-Scnn1b)6608Bouc mice

respiratory system inflammation ( J:191673 )

• airway inflammation levels are similar to single Tg(Scgb1a1-Scnn1b)6608Bouc mice

abnormal mucociliary clearance ( J:191673 )

• intraluminal mucus obstruction is increased compared to single Tg(Scgb1a1-Scnn1b)6608Bouc mice

immune system

respiratory system inflammation ( J:191673 )

• airway inflammation levels are similar to single Tg(Scgb1a1-Scnn1b)6608Bouc mice

cellular

pulmonary epithelial necrosis ( J:191673 )

• airway necrosis is increased compared to single Tg(Scgb1a1-Scnn1b)6608Bouc mice

Genotype
MGI:3801014

cx2

• Allelic Composition: Cftr^tm1Unc/Cftr^tm1Unc; Tg(FABPCFTR)1Jaw/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6 * FVB/N

respiratory system

abnormal alveolar macrophage physiology ( J:129519 )

• cAMP-induced currents CL^- in alveolar macrophages are absent in these mice

• cultured alveolar macrophages are impaired in their ability to kill bacteria that have been phagocytosed

• alveolar macrophages contain 4 times more live intracellular P. aeruginosa bacteria than controls 4 hours after infection

• defective killing of E. Coli in culture also occurs with 3 times more surviving bacteria found two hours post-infection than in controls

• lysosome acidification is defective in alveolar macrophage with pH being at least 1 unit higher than in controls

mortality/aging

mortality/aging ( J:21934 )

N • the high lethality rate of Cftr^tm1Unc homozygotes is prevented by the presence of the Tg(FABPCFTR)1Jaw transgene

digestive/alimentary system

abnormal colon goblet cell morphology ( J:21934 )

• goblet cell hyperplasia occurs in the colon

• transgene expression prevents the goblet cell hyperplasia that occurs in the small intestine of Cftr^tm1Unc homozygotes

abnormal cecum morphology ( J:21934 )

• transgene expression prevents the coiled "worm-like" structure of the cecum that occurs in Cftr^tm1Unc homozygotes

decreased intestinal epithelial chloride transmembrane transport ( J:21934 )

• cyclic-AMP stimulated transport of Cl^- ions does not occur in the large intestine

• cyclic-AMP stimulated transport of Cl^- ions that is absent in the small intestinal epithelium of Cftr^tm1Unc homozygotes is restored in these mice

endocrine/exocrine glands

abnormal colon goblet cell morphology ( J:21934 )

• goblet cell hyperplasia occurs in the colon

• transgene expression prevents the goblet cell hyperplasia that occurs in the small intestine of Cftr^tm1Unc homozygotes

cellular

abnormal colon goblet cell morphology ( J:21934 )

• goblet cell hyperplasia occurs in the colon

• transgene expression prevents the goblet cell hyperplasia that occurs in the small intestine of Cftr^tm1Unc homozygotes