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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Lrp4tm2Her
targeted mutation 2, Joachim Herz
MGI:4455050
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Lrp4tm2Her/Lrp4tm2Her involves: 129S/SvEv MGI:4455052
hm2
Lrp4tm2Her/Lrp4tm2Her involves: 129S/SvEvBrd * C57BL/6J MGI:5561073
hm3
Lrp4tm2Her/Lrp4tm2Her involves: 129S/SvEv * C57BL MGI:5289695


Genotype
MGI:4455052
hm1
Allelic
Composition
Lrp4tm2Her/Lrp4tm2Her
Genetic
Background
involves: 129S/SvEv
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lrp4tm2Her mutation (0 available); any Lrp4 mutation (34 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
skeleton
• exhibit folded enamel and dentin on the labial side
• lingual surfaces of the incisors are also very uneven, with protrusions producing a "corrugated" appearance
• the lingual portion shows a discontinuity in the epithelium and the apical edge resembles an epithelial root sheath normally seen only in developing molar tooth roots
• exhibit supernumerary maxillary incisor tooth germs at birth
• have extra molar cusps

craniofacial
• exhibit folded enamel and dentin on the labial side
• lingual surfaces of the incisors are also very uneven, with protrusions producing a "corrugated" appearance
• the lingual portion shows a discontinuity in the epithelium and the apical edge resembles an epithelial root sheath normally seen only in developing molar tooth roots
• exhibit supernumerary maxillary incisor tooth germs at birth
• have extra molar cusps

growth/size/body
• exhibit folded enamel and dentin on the labial side
• lingual surfaces of the incisors are also very uneven, with protrusions producing a "corrugated" appearance
• the lingual portion shows a discontinuity in the epithelium and the apical edge resembles an epithelial root sheath normally seen only in developing molar tooth roots
• exhibit supernumerary maxillary incisor tooth germs at birth
• have extra molar cusps




Genotype
MGI:5561073
hm2
Allelic
Composition
Lrp4tm2Her/Lrp4tm2Her
Genetic
Background
involves: 129S/SvEvBrd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lrp4tm2Her mutation (0 available); any Lrp4 mutation (34 available)
phenotype observed in females
phenotype observed in males
N normal phenotype



Genotype
MGI:5289695
hm3
Allelic
Composition
Lrp4tm2Her/Lrp4tm2Her
Genetic
Background
involves: 129S/SvEv * C57BL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lrp4tm2Her mutation (0 available); any Lrp4 mutation (34 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Unilateral and bilateral kidney agenesis in Lrp4tm2Her/Lrp4tm2Her mice

mortality/aging
• homozygotes exhibit immediate post-partum lethality due to neuromuscular junction defects

renal/urinary system
• at P0, homozygotes display subpenetrant renal agenesis with rudimentary ureters
• kidney agenesis involves only structures derived from the ureteric bud and metanephric mesenchyme
• the small number of kidneys that do form appear morphologically normal
• the lower urinary and genital systems of both male and female homozygotes remain intact
• at E12.5, the metanephric mesenchyme is absent, unlike in wild-type mice
• 51% of homozygotes display bilateral renal agenesis
• 22% of homozygotes display unilateral renal agenesis
• at P0, homozygotes display bilateral or unilateral renal agenesis with rudimentary ureters
• all newborn homozygotes display at least a partial ureter
• only 12.5% (1/8) of 38 somite stage newborn homozygotes have formed a ureteric bud relative to 100% of wild type controls, indicating a delay in ureteric bud formation
• the ureteric bud fails to undergo secondary branching
• experssion of several branching regulators is lost by E12.5, unlike in wild-type mice
• at E11.5, the ureteric bud fails to invade the metanephric mesenchyme and form a T-shape
• mesenchymal expression of Pax2 is lost by E12.5, unlike in wild-type mice

nervous system
• homozygotes die of neuromuscular junction defects

Mouse Models of Human Disease
OMIM ID Ref(s)
Cenani-Lenz Syndactyly Syndrome; CLSS 212780 J:160547





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last database update
05/17/2016
MGI 6.03
The Jackson Laboratory