Phenotypes Associated with This Genotype

Genotype
MGI:4459095

• Allelic Composition: Tg(Mbp-Pad2)28Fgm/Tg(Mbp-Pad2)28Fgm
• Genetic Background: involves: CD-1

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

nervous system

nervous system phenotype ( J:142650 )

N • no signs of immune cell infiltrates are detected

convulsive seizures ( J:142650 )

• develop convulsions by 1.5 months of age

abnormal astrocyte morphology ( J:142650 )

• thickened astrocytic processes are seen in the optic nerve

abnormal myelin sheath morphology ( J:142650 )

• optic nerves display areas of myelinolysis and thinly myelinated axons

• gene dosage correlated decrease in myelinated fiber density and myelin thickness

• however, no defects in myelin are detected in the peripheral nervous system (assayed in the sciatic nerve)

demyelination ( J:142650 )

• show clinical signs of demyelination (tail droop, shaky gait, head shaking, convulsions, impaired righting response, reduced physical activity) by 1.5 months of age

• signs increase in severity with age more rapidly than in hemizygous mice

• decrease in myelination and irregular axonal staining in the brain

• myelin degradation, nude axons and thinly myelinated axons in the presence of myelinolysis are seen in the optic nerve

behavior/neurological

tremors ( J:142650 )

• develop tremors by 1.5 months of age

abnormal motor coordination/balance ( J:142650 )

• display proprioceptive positioning defects at 4 months of age

• effects are more severe than in hemizygous mice

impaired righting response ( J:142650 )

• becomes impaired by 1.5 months of age

impaired balance ( J:142650 )

• at 4 months of age

• effects are more severe than in hemizygous mice

impaired coordination ( J:142650 )

• at 4 months of age

• effects are more severe than in hemizygous mice

weakness ( J:142650 )

• hind end weakness is seen at 4 months of age

abnormal posture ( J:142650 )

• develop tail droop by 1.5 months of age

head shaking ( J:142650 )

• develops by 1.5 months of age

abnormal gait ( J:142650 )

• develop a shaky gait by 1.5 months of age

• at 4 months of age the gait abnormality is more severe in the hind limbs

convulsive seizures ( J:142650 )

• develop convulsions by 1.5 months of age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
multiple sclerosis		DOID:2377	OMIM:612594 OMIM:612595 OMIM:612596	J:142650