Analysis Tools
behavior/neurological
muscle
|
• muscular atrophy is evident
|
|
• mice exhibit variable signs of muscle weakness such as clenching of the paws, dragging of hind limbs and floppy tail
|
nervous system
|
• sciatic nerves shows widening of myelin lamellae
• poorly compacted areas show absence of the intraperiod line and occasionally, the major dense line is uncompacted, indicating packing abnormalities
|
|
• occasional sciatic nerve fibers show thick and redundant myelin at P28, likely indicating myelin outfolding or tomacula
• more frequent tomacula are seen at P7
• tomacula appear to originate from the paranodal region and contain poorly compacted myelin
|
|
• occasionally older mice show signs of axonal degeneration
|
demyelination
(
J:77658
)
|
• occasionally older mice exhibit demyelinating figures and short internodes suggesting remyelination
• however, no onion bulbs are seen at any age
|
|
• sciatic nerves at P28 show hypomyelination, with thin or absent myelin sheaths on many large caliber axons
• sciatic nerves at 7 months show more pronounced hypomyelination, with most large caliber axons showing very thin or absent myelin
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Charcot-Marie-Tooth disease type 1B | DOID:0110152 |
OMIM:118200 |
J:77658 | |
