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Phenotypes Associated with This Genotype
Genotype
MGI:5577182
Allelic
Composition
Tg(Thy1-FUS*)19Vlb/0
Genetic
Background
B6.Cg-Tg(Thy1-FUS*)19Vlb
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
• mice do not survive past 5 months of age
• after developing the first signs of unstable gait, mice reach the terminal stage of disease within a maximum of 2 weeks

behavior/neurological
• at around 2.5-4.5 months of age, mice abruptly develop severe motor dysfunction
• lose righting reflex and the ability to move freely within several days of onset
• impaired gait caused by asymmetrical pareses and eventual complete paralysis of limbs
• mice first show paresis and eventually complete paralysis of limbs

nervous system
• rapidly developing muscle paralysis and muscle atrophy
• rapidly developing muscle paralysis and muscle atrophy
• loss of spinal motor neurons
• at the symptomatic stage, mice lose approximately half of their spinal motor neurons and the remaining neurons are shrunken and/or chromatolytic
• selective loss of lower motor neuron populations in the brainstem, including in the motor trigeminal, hypoglossal, and facial nuclei but not in the nucleus abducens
• FUS-positive inclusions are seen primarily in the lower motor neuron cell bodies, axons and, in a subset of cells, in the nucleus
• inclusions are also seen in other neurons throughout the nervous system, including upper motor neurons in the motor cortex
• large eosinophilic inclusions are occasionally seen in the spinal cord
• both cytoplasmic and nuclear inclusions in neurons are resistant to proteinase K treatment, however they are not stained by amyloid-detecting dyes Congo Red or thioflavin S
• ubiquitin-positive inclusions of various sizes and morphology are often observed in motor neurons
• however, only a fraction of FUS-positive inclusions are ubiquitinated
• FUS-positive inclusions are seen primarily in the lower motor neuron cell bodies, axons and, in a subset of cells, in the nucleus
• both cytoplasmic and nuclear inclusions in neurons are resistant to proteinase K treatment, however they are not stained by amyloid-detecting dyes Congo Red or thioflavin S
• loss of peripheral nerve fibers
• significant neuronal loss and damage of myelinated fibers in peripheral nerves is only seen at the late, generalized stage of the disease

homeostasis/metabolism
• mice become dehydrated

immune system
• rapidly developing muscle paralysis and muscle atrophy

muscle
• rapidly developing muscle paralysis and muscle atrophy

hematopoietic system
• rapidly developing muscle paralysis and muscle atrophy

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 6 DOID:0060198 OMIM:608030
J:203550


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
08/04/2020
MGI 6.15
The Jackson Laboratory