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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Flcntm1Baba
targeted mutation 1, Masaya Baba
MGI:3774414
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Flcntm1Baba/Flcntm1.1Lss
Tg(CAG-cre/Esr1*)1Lbe/0
involves: 129S1/Sv * 129X1/SvJ MGI:5445163
cn2
Flcntm1Baba/Flcntm1.1Lss
Tg(Cdh16-cre)91Igr/0
involves: C57BL/6 * FVB/N * ICR * SJL MGI:3776087
cn3
Flcntm1Baba/Flcntm1Baba
Fnip1tm1.1Baba/Fnip1tm1.1Baba
Fnip2tm1.1Lss/Fnip2tm1.1Lss
Tg(Cdh16-cre)91Igr/0
involves: C57BL/6 * ICR MGI:5897115


Genotype
MGI:5445163
cn1
Allelic
Composition
Flcntm1Baba/Flcntm1.1Lss
Tg(CAG-cre/Esr1*)1Lbe/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Flcntm1.1Lss mutation (0 available); any Flcn mutation (9 available)
Flcntm1Baba mutation (0 available); any Flcn mutation (9 available)
Tg(CAG-cre/Esr1*)1Lbe mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• pro-B cell block in the bone marrow following tamoxifen-treatment
• CD19+ cells in the spleen following tamoxifen-treatment

renal/urinary system
• in tamoxifen-treated mice
• in tamoxifen-treated mice however, kidney phenotype is rescued by rapamycin treatment
• in tamoxifen-treated mice
• however, kidney phenotype is rescued by rapamycin treatment

hematopoietic system
• pro-B cell block in the bone marrow following tamoxifen-treatment
• CD19+ cells in the spleen following tamoxifen-treatment




Genotype
MGI:3776087
cn2
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 (9 available)
Flcntm1Baba mutation (0 available); any Flcn mutation (9 available)
Tg(Cdh16-cre)91Igr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• treatment of mice with rapamycin extends lifespan nearly 2-fold, but all animals die of renal failure
• animals appear normal at birth, but develop distended abdomens and die around 3 weeks of age from renal failure

growth/size/body
• by 2 weeks, mice display distended abdomens which are very pronounced at time of death
• at time of death, enlarged kidneys fill abdominal cavity; penetrance of phenotype is 100%
• enlargement begins at 1 week due to collecting duct dilation
• between P7 and P21, kidney to body weight ratio (under wet and dried conditions) dramatically increases relative to littermate controls

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

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

homeostasis/metabolism
• levels are significantly elevated at 2 and 3 weeks of age, compared to controls

cellular
• in culture, tubule cells show much higher proliferation rate than control cells

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Birt-Hogg-Dube syndrome DOID:0050676 OMIM:135150
J:130978




Genotype
MGI:5897115
cn3
Allelic
Composition
Flcntm1Baba/Flcntm1Baba
Fnip1tm1.1Baba/Fnip1tm1.1Baba
Fnip2tm1.1Lss/Fnip2tm1.1Lss
Tg(Cdh16-cre)91Igr/0
Genetic
Background
involves: C57BL/6 * ICR
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Flcntm1Baba mutation (0 available); any Flcn mutation (9 available)
Fnip1tm1.1Baba mutation (0 available); any Fnip1 mutation (39 available)
Fnip2tm1.1Lss mutation (0 available); any Fnip2 mutation (41 available)
Tg(Cdh16-cre)91Igr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• no increase in severity of phenotype compared to mutant mice wild-type for Flcn

renal/urinary system
• no increase in severity of phenotype compared to mutant mice wild-type for Flcn
• no increase in severity of cystic phenotype compared to mutant mice wild-type for Flcn





Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
04/13/2021
MGI 6.16
The Jackson Laboratory