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Phenotypes Associated with This Genotype
Genotype
MGI:3776087
Allelic
Composition
Flcntm1Baba/Flcntm1.1Lss
Tg(Cdh16-cre)91Igr/0
Genetic
Background
involves: C57BL/6 * FVB/N * ICR * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Flcntm1.1Lss mutation (0 available); any Flcn mutation (10 available)
Flcntm1Baba mutation (0 available); any Flcn mutation (10 available)
Tg(Cdh16-cre)91Igr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• animals appear normal at birth, but develop distended abdomens and die around 3 weeks of age from renal failure (J:130978)
• animals appear normal at birth, but develop distended abdomens and die around 3 weeks of age from renal failure (J:130978)
• treatment of mice with rapamycin extends lifespan nearly 2-fold, but all animals die of renal failure (J:130978)
• treatment of mice with rapamycin extends lifespan nearly 2-fold, but all animals die of renal failure (J:130978)

growth/size/body
• by 2 weeks, mice display distended abdomens which are very pronounced at time of death (J:130978)
• by 2 weeks, mice display distended abdomens which are very pronounced at time of death (J:130978)

renal/urinary system
• kidneys have fine reticular pattern of interstitial tissue containing numerous blood vessels, not observed in control kidneys (J:130978)
• kidneys have fine reticular pattern of interstitial tissue containing numerous blood vessels, not observed in control kidneys (J:130978)
• in culture, tubule cells show much higher proliferation rate than control cells (J:130978)
• in culture, tubule cells show much higher proliferation rate than control cells (J:130978)
• by 2 weeks, lumens of ducts are cystic (J:130978)
• at 3 weeks, collecting ducts in the medulla and extending into the papilla are severely cystic (J:130978)
• by 2 weeks, lumens of ducts are cystic (J:130978)
• at 3 weeks, collecting ducts in the medulla and extending into the papilla are severely cystic (J:130978)
• by 1 week of age, dilation of collecting ducts is observed (J:130978)
• by 1 week of age, dilation of collecting ducts is observed (J:130978)
• anatomical distinction between cortex and medulla is disrupted (J:130978)
• anatomical distinction between cortex and medulla is disrupted (J:130978)
• regions of pyramidal infarctions are observed at 3 weeks (J:130978)
• regions of pyramidal infarctions are observed at 3 weeks (J:130978)
• at time of death, enlarged kidneys fill abdominal cavity; penetrance of phenotype is 100% (J:130978)
• enlargement begins at 1 week due to collecting duct dilation (J:130978)
• at time of death, enlarged kidneys fill abdominal cavity; penetrance of phenotype is 100% (J:130978)
• enlargement begins at 1 week due to collecting duct dilation (J:130978)
• between P7 and P21, kidney to body weight ratio (under wet and dried conditions) dramatically increases relative to littermate controls (J:130978)
• between P7 and P21, kidney to body weight ratio (under wet and dried conditions) dramatically increases relative to littermate controls (J:130978)
• by 2 weeks, lumens of tubules are cystic (J:130978)
• most cells lining the tubules are hypertrophic with enlarged nuclei and cytoplasm; many cells ar hyperplastic (J:130978)
• by 2 weeks, lumens of tubules are cystic (J:130978)
• most cells lining the tubules are hypertrophic with enlarged nuclei and cytoplasm; many cells ar hyperplastic (J:130978)
• by 2 weeks, lumens of tubules are cystic (J:130978)
• by 2 weeks, lumens of tubules are cystic (J:130978)
• at 3 weeks, kidneys are markedly cystic (J:130978)
• at 3 weeks, kidneys are markedly cystic (J:130978)
• occurs around 3 weeks of age (J:130978)
• occurs around 3 weeks of age (J:130978)

cardiovascular system
• kidneys have fine reticular pattern of interstitial tissue containing numerous blood vessels, not observed in control kidneys (J:130978)
• kidneys have fine reticular pattern of interstitial tissue containing numerous blood vessels, not observed in control kidneys (J:130978)

homeostasis/metabolism
• levels are significantly elevated at 2 and 3 weeks of age, compared to controls (J:130978)
• levels are significantly elevated at 2 and 3 weeks of age, compared to controls (J:130978)

cellular
• in culture, tubule cells show much higher proliferation rate than control cells (J:130978)
• in culture, tubule cells show much higher proliferation rate than control cells (J:130978)

Mouse Models of Human Disease
OMIM ID Ref(s)
Birt-Hogg-Dube Syndrome; BHD 135150 J:130978


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory