nervous system phenotype

• slow Wallerian degeneration
  • 5 days after severing the sciatic nerve, mice show slow Wallerian degeneration evidenced by an intact cytoskeleton and plasma membrane, while control mice show a destruction of cytoarchitecture in the distal portion of the severed nerve
  (MGI Ref ID J:19951)(MGI Ref ID J:15229)
  • following optic nerve transection, the axons of retinal ganglion cells undergo very slow Wallerian degeneration
  (MGI Ref ID J:19950)
  • invasion by polymorphs and macrophages of the distal portion of the severed nerve is not observed in mutant mice, resulting in an absence of myelin breakdown; controls show an increase in myeloperoxidase positive cells and Schwann cell proliferation within 3 days of section
  (MGI Ref ID J:19951)
  • 1 year old C57BL/Ola mice showed faster Wallerian degeneration than 4 week old mice, with compund action potentials recorded in a distal nerve stump 5 days after section in 4 week old mutant mice, but not 1 year old mutant mice
  (MGI Ref ID J:3359)
  • in addition, the distal portions of mutant severed nerves show an ability to conduct action potentials up to 14 days post section, while control nerves stop conducting 2 days after the nerve is cut
  (MGI Ref ID J:19951)
  • n 3 days of section
  (MGI Ref ID J:19951)

• abnormal peripheral nervous system regeneration
  • the rate of regeneration and recovery of function after sciatic nerve crush is delayed and incomplete compared to C57BL/6J mice, as determined by sciatic functional index (SFI) using measurments of walking ability and by measurments of axonal transport
  (MGI Ref ID J:12834)
  • the rate of regeneration and recovery of motor function in the soleus muscle after a crush of the sciatic nerve is similar in mutant mice and controls, with a slight delay in recovery of normal conduction velocity in mutant mice
  (MGI Ref ID J:19951)
  • the rate of regeneration and recovery of function after facial nerve crush is delayed compared to C57BL/6J mice, as observed by vibrissae movement
  (MGI Ref ID J:12834)

• retinal ganglion cell degeneration
  • while retinal ganglion cells undergo retrograde degeneration in response to optic nerve injury, they do so at a much slower rate than in other strains
  (MGI Ref ID J:19950)

vision/eye phenotype

• retinal ganglion cell degeneration
  • while retinal ganglion cells undergo retrograde degeneration in response to optic nerve injury, they do so at a much slower rate than in other strains
  (MGI Ref ID J:19950)

homeostasis/metabolism phenotype

• slow Wallerian degeneration
  • n 3 days of section
  (MGI Ref ID J:19951)(MGI Ref ID J:15229)
  • 5 days after severing the sciatic nerve, mice show slow Wallerian degeneration evidenced by an intact cytoskeleton and plasma membrane, while control mice show a destruction of cytoarchitecture in the distal portion of the severed nerve
  (MGI Ref ID J:19951)
  • in addition, the distal portions of mutant severed nerves show an ability to conduct action potentials up to 14 days post section, while control nerves stop conducting 2 days after the nerve is cut
  (MGI Ref ID J:19951)
  • 1 year old C57BL/Ola mice showed faster Wallerian degeneration than 4 week old mice, with compund action potentials recorded in a distal nerve stump 5 days after section in 4 week old mutant mice, but not 1 year old mutant mice
  (MGI Ref ID J:3359)
  • following optic nerve transection, the axons of retinal ganglion cells undergo very slow Wallerian degeneration
  (MGI Ref ID J:19950)
  • invasion by polymorphs and macrophages of the distal portion of the severed nerve is not observed in mutant mice, resulting in an absence of myelin breakdown; controls show an increase in myeloperoxidase positive cells and Schwann cell proliferation within 3 days of section
  (MGI Ref ID J:19951)