Phenotypes Associated with This Genotype

Genotype
MGI:3513395

• Allelic Composition: Prx^tm1Brp/Prx^tm1Brp
• Genetic Background: involves: 129P2/Ola * C57BL/6

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

behavior/neurological

aphagia ( J:62566 )

• inability to eat leading to rapid weight loss

limb grasping ( J:62566 )

• hindlimb clasping when lifted by the tail is seen between the ages of 4 and 6 weeks with slight tremors as well in some animals

abnormal gait ( J:62566 )

• unsteady gait by 6-9 months of age

• difficulty supporting themselves on their hind limbs

abnormal touch/ nociception ( J:62566 )

hyporesponsive to tactile stimuli ( J:62566 )

• lower threshold for response using von Frey hairs on hind paws

decreased thermal nociceptive threshold ( J:62566 )

• lower thermal nociceptive thresholds

growth/size/body

weight loss ( J:62566 )

• rapid weight loss due to inability to eat

respiratory system

abnormal respiratory mechanics ( J:62566 )

• labored breathing

nervous system

abnormal Schwann cell morphology ( J:92662 )

• growth rate of schwann cells is lower than the extension rate of their axons

abnormal myelination ( J:62566 )

• at 6 weeks of age, sciatic nerves display focal thickening and infoldings of internodal myelin

• demyelination

• by 6 months of age, extensive demyelination has occurred in sensory, motor, and autonomic nerves with focal thickening in internodes and axonal sheath disruption

• unmyelinated C fiber bundles are morphologically normal

• remyelination after injury is retarded even in younger mice

abnormal nervous system electrophysiology ( J:62566 )

abnormal action potential ( J:62566 )

• reduced action potentials

abnormal nerve conduction ( J:62566 )

• reduced conduction velocity