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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp-FTL*)4Ruvi
transgene insertion 4, Ruben Vidal
MGI:3848965
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Prnp-FTL*)4Ruvi/Tg(Prnp-FTL*)4Ruvi B6.C3Fe-Tg(Prnp-FTL*)4Ruvi MGI:3848974
tg2
Tg(Prnp-FTL*)4Ruvi/0 B6.C3Fe-Tg(Prnp-FTL*)4Ruvi MGI:3848973


Genotype
MGI:3848974
tg1
Allelic
Composition
Tg(Prnp-FTL*)4Ruvi/Tg(Prnp-FTL*)4Ruvi
Genetic
Background
B6.C3Fe-Tg(Prnp-FTL*)4Ruvi
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• survival rate is decreased by 36%
• survival rate is decreased by 36%

behavior/neurological
• at 4 months, mice exhibit decreased latency to fall on a rotarod compared with wild-type mice
• towards the end of their lives
• late in life mice exhibit a shuffle gait with dragging hindlimbs

nervous system
• ferritin nuclear inclusions are observed in Purkinje cells and granule cell layer neurons unlike in wild-type mice
• ferritin nuclear inclusions are observed in Purkinje cells and granule cell layer neurons unlike in wild-type mice
• mice exhibit an age-dependent increase in the size of ferritin nuclear inclusions and the number of cells with nuclear inclusions compared to in wild-type mice
• mice exhibit ferritin inclusions in the substantia nigra, striatum, amygdala, cerebral cortex, and Purkinje cells and granule cells of the cerebellum unlike in wild-type mice

homeostasis/metabolism
• ferritin inclusions are observed in neural tissues; cardiac cells; striated muscle of the tongue; mucosa, muscularis mucosae, submucosa and muscularis external layers of the gastrointestinal tract; hepatocytes of the liver; adrenal glands and adjacent adipose tissue; cuboidal cells of the convolute tubules of the kidneys; connective tissue of the urinary bladder; ureters; cuboidal cells of the excretory ducts and fibroblasts of the reticular layer of the skin; mononuclear cells of lymphoid nodules and endothelial cells of blood vessels of the spleen; striated and smooth muscle tissues; epithelium of uterine glands in females; seminiferous tubes of the testis, intratesticular genitals ducts and epididymidis in males; and alveolar epithelial cells and smooth muscle cells of the lungs

skeleton
• mice exhibit a very low body position with some degree of lordosis unlike wild-type mice

Mouse Models of Human Disease
OMIM ID Ref(s)
Neurodegeneration with Brain Iron Accumulation 3; NBIA3 606159 J:131053




Genotype
MGI:3848973
tg2
Allelic
Composition
Tg(Prnp-FTL*)4Ruvi/0
Genetic
Background
B6.C3Fe-Tg(Prnp-FTL*)4Ruvi
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• survival rate is decreased by 15%
• median survival is 640 days

nervous system
• mice exhibit granule cell degeneration
• mice exhibit an age-dependent increase in the size of ferritin nuclear inclusions and the number of cells with nuclear inclusions compared to in wild-type mice
• ferritin inclusion bodies are found in neurons of the striatum, cerebellum, olfactory bulb, Purkinje cells, granule cells, gray matter of the spinal cord, and choroid plexus unlike in wild-type mice

homeostasis/metabolism
• ferritin inclusions are observed in neural tissues; cardiac cells; striated muscle of the tongue; mucosa, muscularis mucosae, submucosa and muscularis external layers of the gastrointestinal tract; hepatocytes of the liver; adrenal glands and adjacent adipose tissue; cuboidal cells of the convolute tubules of the kidneys; connective tissue of the urinary bladder; ureters; cuboidal cells of the excretory ducts and fibroblasts of the reticular layer of the skin; mononuclear cells of lymphoid nodules and endothelial cells of blood vessels of the spleen; striated and smooth muscle tissues; epithelium of uterine glands in females; seminiferous tubes of the testis, intratesticular genitals ducts and epididymidis in males; and alveolar epithelial cells and smooth muscle cells of the lungs





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last database update
06/22/2016
MGI 6.04
The Jackson Laboratory