Phenotypes associated with this allele

• Allele Symbol: Mbp^jve
• Allele Name: jive
• Allele ID: MGI:3708458
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Mbp^jve/Mbp^jve	involves: C57BL/6J	MGI:3709055

Genotype
MGI:3709055

hm1

• Allelic Composition: Mbp^jve/Mbp^jve
• Genetic Background: involves: C57BL/6J

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:221049 )

• premature death starting around 75 days of age, with all mice dead by 3 months due to progressive increase in seizure intensity

growth/size/body

decreased body weight ( J:221049 )

• significantly decreased body weight in males at 4, 5, and 8 weeks of age and in females only at 8 weeks of age

behavior/neurological

decreased startle reflex ( J:119820 )

• low auditory startle response

tremors ( J:221049 )

• a severe shaking phenotype is apparent before weaning at 3 weeks of age

• shaking occurs upon locomotion

abnormal gait ( J:221049 )

• distinct jive dance-like gait

decreased locomotor activity ( J:221049 )

• significant reduction in average distance moved during a 6-min open field test relative to wild-type controls around 50 days of age

• however, no differences in mobility or time spent in the periphery, center or corners of the open field

environmentally induced seizures ( J:221049 )

• seizures occur upon external stimuli such as cage opening and researcher interference in the cage

tonic seizures ( J:221049 )

• silent seizures progress into tonic seizures

absence seizures ( J:221049 )

• shaking is followed by silent seizures around 50 days of age

nervous system

environmentally induced seizures ( J:221049 )

• seizures occur upon external stimuli such as cage opening and researcher interference in the cage

tonic seizures ( J:221049 )

• silent seizures progress into tonic seizures

absence seizures ( J:221049 )

• shaking is followed by silent seizures around 50 days of age

abnormal brain white matter morphology ( J:221049 )

• severe alterations in white matter structures at 45 and 65 days of age, as detected by T1, T2 and diffusion weighted magnetic resonance imaging

• however, no significant alterations in grey matter structures such as the cortex and hippocampus

abnormal corpus callosum morphology ( J:221049 )

• increase in signal intensity in the area of corpus callosum on T2-weighted images at 45 and 65 days of age, with decrease in signal intensity seen on T1-weighted images at 45 days

• significant increase in spin-spin relaxation time (T2) in the corpus callosum at 45 and 65 days of age

abnormal cerebellum white matter morphology ( J:221049 )

• decrease in signal intensity in the area of cerebellum seen on T1-weighted images at 45 days of age

abnormal action potential ( J:221049 )

• slight reduction in sensory nerve action potential (SNAP) latency relative to wild-type controls over the musculus gastrocnemius at 8 weeks but not at 4 or 5 weeks of age

• however, no differences in SNAP amplitude, compound muscle action potential (CMAP) latency and CMAP amplitude relative to wild-type controls at 4, 5 and 8 weeks of age, suggesting that peripheral nerve conductance is largely normal

hearing/vestibular/ear

increased or absent threshold for auditory brainstem response ( J:119820 )

• auditory threshold of more than 90 dB, indicating that lines are severely hearing impaired in 8- to 12-week-old animals