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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 (34 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)

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
• the remaining pancreatic cilia are shortened at E15.5, E18.5 and 4 days of age
• pancreatic primary cilia are almost absent
• progressive loss of acinar cells from birth to 17 days
• relative to acinar and beta-cells a 3-fold increase in ductal cell proliferation is seen
• loss of acinar cells results in severe reduction in pancreatic mass
• by 2 weeks of age large pancreatic cyst is seen and at E18.5, intracinar ducts are slightly dilated
• shortly after birth amylase expression is increased; however glucose tolerance is normal

limbs/digits/tail
• at E12.5, minor expansion of the limb bud is seen mainly on the anterior side
• carpals are fused or malformed in many mice
• phalange 2 of digits II and V is severely reduced in length or absent
• 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
• tarsals are fused or malformed in many mice

craniofacial
• the parietal and frontal sutures are disorganized and fail to fuse
• 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
• minor clefting of the palatine process

hearing/vestibular/ear
• the opening in the perioctic capsule is absent

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

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

embryo
• at E12.5, minor expansion of the limb bud is seen mainly on the anterior side

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)
• the remaining pancreatic cilia are shortened at E15.5, E18.5 and 4 days of age
• pancreatic primary cilia are almost absent

growth/size/body
• 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
• minor clefting of the palatine process
• by 2 weeks of age large pancreatic cyst is seen and at E18.5, intracinar ducts are slightly dilated

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
autosomal recessive polycystic kidney disease DOID:0110861 OMIM:263200
J:65500 , J:90990


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
09/22/2022
MGI 6.21
The Jackson Laboratory