behavior/neurological phenotype

• ataxia
  (MGI Ref ID J:38856)

• tremors
  (MGI Ref ID J:38856)

mortality/aging

• premature death
  • die around 3 months of age
  (MGI Ref ID J:38856)

nervous system phenotype

• abnormal myelin sheath morphology
  • when present axon sheaths are thinner than normal and poorly compacted
  (MGI Ref ID J:38856)

• abnormal myelination
  • dysmyelination with many naked axons
  (MGI Ref ID J:38856)

behavior/neurological phenotype

• abnormal maternal nurturing
  • clinical manifestations interfere with normal mothering
  (MGI Ref ID J:6578)

• abnormal spatial learning
  • in a T-maze, mice exhibit impaired successive reversal learning compared with wild-type mice
  (MGI Ref ID J:27482)

homeostasis/metabolism phenotype

• decreased brain cholesterol level
  (MGI Ref ID J:9907)

• decreased cholesterol level
  • slightly in the sciatic nerve
  (MGI Ref ID J:9907)

mammalian phenotype

• reproductive system phenotype
  • Normal - mice are fertile
  (MGI Ref ID J:6578)

mortality/aging

• premature death
  • mice die between P50 and P100
  (MGI Ref ID J:6578)
  • Background Sensitivity - mice on a pure SWV background die sooner than mice on a mixed C3H, ICR, and SWV background
  (MGI Ref ID J:6578)

nervous system phenotype

• decreased brain cholesterol level
  (MGI Ref ID J:9907)

behavior/neurological phenotype

• audiogenic seizures
  (MGI Ref ID J:6578)

• environmentally induced seizures
  • seizures can be triggered by sound, motion, light, and handling
  (MGI Ref ID J:6578)

• hindlimb paralysis
  • on rare occasion with age
  (MGI Ref ID J:6578)

• tonic seizures
  • from P30
  (MGI Ref ID J:6578)

• tremors
  • beginning at 12 days when animals are active
  (MGI Ref ID J:6578)

mortality/aging

• premature death
  • mice die between P50 and P100
  (MGI Ref ID J:6578)
  • Background Sensitivity - mice on a mixed C3H, ICR, and SWV background live longer than mice on a pure SWV background
  (MGI Ref ID J:6578)

nervous system phenotype

• audiogenic seizures
  (MGI Ref ID J:6578)

• environmentally induced seizures
  • seizures can be triggered by sound, motion, light, and handling
  (MGI Ref ID J:6578)

• tonic seizures
  • from P30
  (MGI Ref ID J:6578)

behavior/neurological phenotype

• impaired coordination
  • Normal - however, transgenic expression of Mbp or transplantation of wild-type oligodendrocytes improve performance on a rotarod
  (MGI Ref ID J:127688)
  • on a rotarod, mice exhibit impaired coordination compared with wild-type mice
  (MGI Ref ID J:127688)

nervous system phenotype

• abnormal axonal transport
  • increased
  (MGI Ref ID J:57128)

• abnormal myelination
  • mice lack compact myelin unlike wild-type mice
  (MGI Ref ID J:57128)

• abnormal neuron morphology
  • neurons exhibit increased microtubule number and density and neurofilament density compared with wild-type cells
  (MGI Ref ID J:57128)

cellular phenotype

• increased T cell proliferation
  • T cells primed with MBP proliferate in culture unlike wild-type cells
  (MGI Ref ID J:48412)

hematopoietic system phenotype

• increased T cell proliferation
  • T cells primed with MBP proliferate in culture unlike wild-type cells
  (MGI Ref ID J:48412)

immune system phenotype

• increased interferon-gamma secretion
  • when primed with MBP
  (MGI Ref ID J:48412)

• increased interleukin-2 secretion
  • when primed with MBP
  (MGI Ref ID J:48412)

• increased interleukin-4 secretion
  • when primed with MBP
  (MGI Ref ID J:48412)

• increased susceptibility to experimental autoimmune encephalomyelitis
  • following induction of experimental autoimmune encephalomyelitis (EAE), mice develop moderate to severe EAE unlike similarly treated wild-type mice
  (MGI Ref ID J:48412)

• increased T cell proliferation
  • T cells primed with MBP proliferate in culture unlike wild-type cells
  (MGI Ref ID J:48412)

hearing/vestibular/ear phenotype

• absent linear vestibular evoked potential
  • in one of two mice tested, VESPs are absent at the maximum stimulus intensity used
  (MGI Ref ID J:116914)

• reduced linear vestibular evoked potential
  • elevated threshold and absent central response peaks in aged mice
  (MGI Ref ID J:116914)

behavior/neurological phenotype

• limb grasping
  • mice show severe hind limb clasping at P56
  (MGI Ref ID J:211344)

• tremors
  • mice develop a severe generalized tremor during locomotion by 2 weeks of age, indicating CNS defects
  (MGI Ref ID J:211344)

hearing/vestibular/ear phenotype

• reduced linear vestibular evoked potential
  • elevated threshold for aged mice and prolonged latency for central response peaks in young and aged mice
  (MGI Ref ID J:116914)

immune system phenotype

• decreased susceptibility to viral infection
  • following infection with Theiler's murine encephalomyelitis virus (TMEV), mice exhibit no viral RNA or signs of infection in the spinal cord unlike similarly treated wild-type mice
  (MGI Ref ID J:41447)

mammalian phenotype

• immune system phenotype
  • Normal - T cell, B cell, and macrophage counts are normal
  (MGI Ref ID J:41447)

nervous system phenotype

• abnormal myelin sheath morphology
  • Normal - however, no major structural defects in PNS myelination are detected at P10 or P56 and the distribution of Na_v- and K_v1.2-channels in teased sciatic nerve fibers is normal at P56, indicating that the structure of the node of Ranvier is preserved
  (MGI Ref ID J:211344)
  • mice show a significant increase in the number of Schmidt-Lanterman incisures on sciatic nerve cross sections relative to control mice
  (MGI Ref ID J:211344)

• decreased nerve conduction velocity
  • at P56, mice show a significant reduction in motor nerve conduction velocity relative to control mice
  (MGI Ref ID J:211344)