mortality/aging
• died usually 3-8 weeks after birth
|
nervous system
• neurodystrophic changes with numerous spheroid bodies in nervous tissues such as in the hippocampus, thalamus, hypothalamus, pons, callosal body, striatum, globus pallidus, and the cervical, thoracic and lumbar vertebrae
|
• axonal dystrophic change represented by spheroid bodies in the gracile tract of the medulla oblongata
|
• hippocampus is hardly distinguishable due to its thinness and caudal displacement caused by ventricular enlargement
|
behavior/neurological
limb grasping
(
J:97670
)
• when suspended by tail, the limbs become flexed and then fold toward the trunk compared to controls in which limbs are held extended out from the trunk
|
• progressive motor paralysis and atrophy of the hindlimbs, which show muscle weakness with foot-droop
|
paraparesis
(
J:97670
)
• dragging of hindlimbs
|
limbs/digits/tail
• initially mutants exhibit an abnormal limb posture and later show atrophy of the hindlimbs
|
adipose tissue
• lean body, particularly in the lower half
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
neurodegeneration with brain iron accumulation 2a | DOID:0110735 |
OMIM:256600 |
J:97670 |