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Phenotypes Associated with This Genotype
Genotype
MGI:3046284
Allelic
Composition
Ift88Tg737Rpw/Ift88Tg737Rpw
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ift88Tg737Rpw mutation (0 available); any Ift88 mutation (5 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• in tubules distal to the proximal tubules, cilia are shorter compared to wild-type cells (1.0+/-0.6 and 1.3+/-0.6 compared to 3.1+/-1.4 and 3.5+/-1.7 at 4 and 7 days of age, respectively) (J:65500)
• in tubules distal to the proximal tubules, cilia are shorter compared to wild-type cells (1.0+/-0.6 and 1.3+/-0.6 compared to 3.1+/-1.4 and 3.5+/-1.7 at 4 and 7 days of age, respectively) (J:65500)

endocrine/exocrine glands
• increased dilation of duct-like structures and pancreatic lobules are separated by increased interstitial space as well as increased number of tubular complexes; however the islets of Langerhans are morphologically normal and not reduced in number (J:90990)
• primary cilia are almost absent and the remaining cilia are shortened at E15.5, E18.5 and 4 days of age (J:90990)
• increased dilation of duct-like structures and pancreatic lobules are separated by increased interstitial space as well as increased number of tubular complexes; however the islets of Langerhans are morphologically normal and not reduced in number (J:90990)
• primary cilia are almost absent and the remaining cilia are shortened at E15.5, E18.5 and 4 days of age (J:90990)
• progressive loss of acinar cells from birth to 17 days (J:90990)
• progressive loss of acinar cells from birth to 17 days (J:90990)
• relative to acinar and beta-cells a 3-fold increase in ductal cell proliferation is seen (J:90990)
• relative to acinar and beta-cells a 3-fold increase in ductal cell proliferation is seen (J:90990)
• loss of acinar cells results in severe reduction in pancreatic mass (J:90990)
• loss of acinar cells results in severe reduction in pancreatic mass (J:90990)
• by 2 weeks of age large pancreatic cyst is seen and at E18.5, intracinar ducts are slightly dilated (J:90990)
• by 2 weeks of age large pancreatic cyst is seen and at E18.5, intracinar ducts are slightly dilated (J:90990)
• shortly after birth amylase expression is increased; however glucose tolerance is normal (J:90990)
• shortly after birth amylase expression is increased; however glucose tolerance is normal (J:90990)

limbs/digits/tail
• at E12.5, minor expansion of the limb bud is seen mainly on the anterior side (J:83305)
• at E12.5, minor expansion of the limb bud is seen mainly on the anterior side (J:83305)
• phalange 2 of digits II and V is severely reduced in length or absent (J:83305)
• phalange 2 of digits II and V is severely reduced in length or absent (J:83305)
• in the forelimbs polydactyly is often seen as a fusion with digit I and in the hindlimbs polydactyly is a complete duplication with both phalanges resembling digit I (J:83305)
• in the forelimbs polydactyly is often seen as a fusion with digit I and in the hindlimbs polydactyly is a complete duplication with both phalanges resembling digit I (J:83305)
• tarsals are fused or malformed in many mice (J:83305)
• tarsals are fused or malformed in many mice (J:83305)
• carpals are fused or malformed in many mice (J:83305)
• carpals are fused or malformed in many mice (J:83305)

craniofacial
• the parietal and frontal sutures are disorganized and fail to fuse (J:83305)
• the parietal and frontal sutures are disorganized and fail to fuse (J:83305)
• minor clefting of the palatine process (J:83305)
• minor clefting of the palatine process (J:83305)
• mice have 4 rather than 3 molars on both the upper and lower jaws with the extra molar composed of a single cuspid and rostral to what is normally the first molar (J:83305)
• mice have 4 rather than 3 molars on both the upper and lower jaws with the extra molar composed of a single cuspid and rostral to what is normally the first molar (J:83305)
• mice have 4 rather than 3 molars on both the upper and lower jaws with the extra molar composed of a single cuspid and rostral to what is normally the first molar (J:83305)
• mice have 4 rather than 3 molars on both the upper and lower jaws with the extra molar composed of a single cuspid and rostral to what is normally the first molar (J:83305)

hearing/vestibular/ear
• the opening in the perioctic capsule is absent (J:83305)
• the opening in the perioctic capsule is absent (J:83305)

skeleton
• the parietal and frontal sutures are disorganized and fail to fuse (J:83305)
• the parietal and frontal sutures are disorganized and fail to fuse (J:83305)
• tarsals are fused or malformed in many mice (J:83305)
• tarsals are fused or malformed in many mice (J:83305)
• carpals are fused or malformed in many mice (J:83305)
• carpals are fused or malformed in many mice (J:83305)
• the tibia and fibula fail to fuse (J:83305)
• the tibia and fibula fail to fuse (J:83305)
• phalange 2 of digits II and V is severely reduced in length or absent (J:83305)
• phalange 2 of digits II and V is severely reduced in length or absent (J:83305)

digestive/alimentary system
• minor clefting of the palatine process (J:83305)
• minor clefting of the palatine process (J:83305)
• progressive loss of acinar cells from birth to 17 days (J:90990)
• progressive loss of acinar cells from birth to 17 days (J:90990)
• relative to acinar and beta-cells a 3-fold increase in ductal cell proliferation is seen (J:90990)
• relative to acinar and beta-cells a 3-fold increase in ductal cell proliferation is seen (J:90990)

embryogenesis
• at E12.5, minor expansion of the limb bud is seen mainly on the anterior side (J:83305)
• at E12.5, minor expansion of the limb bud is seen mainly on the anterior side (J:83305)

cellular
• in tubules distal to the proximal tubules, cilia are shorter compared to wild-type cells (1.0+/-0.6 and 1.3+/-0.6 compared to 3.1+/-1.4 and 3.5+/-1.7 at 4 and 7 days of age, respectively) (J:65500)
• in tubules distal to the proximal tubules, cilia are shorter compared to wild-type cells (1.0+/-0.6 and 1.3+/-0.6 compared to 3.1+/-1.4 and 3.5+/-1.7 at 4 and 7 days of age, respectively) (J:65500)

growth/size/body
• minor clefting of the palatine process (J:83305)
• minor clefting of the palatine process (J:83305)
• mice have 4 rather than 3 molars on both the upper and lower jaws with the extra molar composed of a single cuspid and rostral to what is normally the first molar (J:83305)
• mice have 4 rather than 3 molars on both the upper and lower jaws with the extra molar composed of a single cuspid and rostral to what is normally the first molar (J:83305)
• mice have 4 rather than 3 molars on both the upper and lower jaws with the extra molar composed of a single cuspid and rostral to what is normally the first molar (J:83305)
• mice have 4 rather than 3 molars on both the upper and lower jaws with the extra molar composed of a single cuspid and rostral to what is normally the first molar (J:83305)

Mouse Models of Human Disease
OMIM ID Ref(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:65500 , J:90990


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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