Mouse Genome Informatics
hm1
    Nefltm1Jpj/Nefltm1Jpj
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
• intermediate filament structures scarce in the axons of adults but present in new born mice
• density of microtubules increased dramatically
• large myelinated fibers of much smaller diameter than normal
• leads to a unimodal rather than bimodal size distribution
• myelin sheaths are normal even though the axonal diameter is smaller
• after sciatic or facial nerve crush, the number of regenerating axons was considerably reduced
• considerable growth was seen by 2 months after nerve crush however
• axonal profile is similar to NF-H/NF-M mutants; reduction in ventral root axonal caliber is similar


Mouse Genome Informatics
cx2
    Nefltm1Jpj/Nefltm1Jpj
Tg(SOD1*G85R)148Dwc/0

involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
• at 12 months of age, no axon loss or degeneration in motor nerves
• mice start out with a 13% reduction of motor neurons
• further degeneration is delayed but ultimately reaches the same level of motor axon loss
• loss of axons in sensory neurons is increased
• slower development of ALS symptoms than in the presence of the transgene alone