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Phenotypes Associated with This Genotype
Genotype
MGI:5577168
Allelic
Composition
DstTg4/DstTg4
Genetic
Background
involves: C57BL/6 * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
DstTg4 mutation (0 available); any Dst mutation (175 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die at around P20

growth/size/body
• at around 2 weeks of age, mice show an arrest in weight-gain

behavior/neurological
• hind limb clasping is seen at P15 or earlier
• mice fall off a beam and are unable to effectively balance and/or grip to remain on the suspended beam
• at around 2 weeks of age, mice show ambulating abnormalities and at P20, mice exhibit an aberrant gait

nervous system
• degeneration of muscle spindles in tibialis anterior muscles
• microtubule network is disorganized, with an accumulation of beta-III tubulin throughout axons
• dilated rough ER sheets and Golgi membranes in P5 sensory neurons
• increase in the number of immature endplates and neuromuscular junctions in muscles
• sensory neuron degeneration begins at P15
• dorsal sensory roots are smaller, have fewer axons, axons undergoing degeneration and axonal swellings
• dorsal sensory roots show axons undergoing degeneration

immune system
• P20 mice occasionally exhibit conjunctivitis

muscle
• degeneration of muscle spindles in tibialis anterior muscles

vision/eye
• P20 mice occasionally exhibit conjunctivitis
• P20 mice occasionally exhibit blepharoptosis

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
hereditary sensory neuropathy DOID:0050548 OMIM:PS162400
J:209161


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
01/12/2021
MGI 6.16
The Jackson Laboratory