Phenotypes associated with this allele

• Allele Symbol: Tg(Gria2*586N)238.2Rsp
• Allele Name: transgene insertion 238.2, Rolf Sprengel
• Allele ID: MGI:3612450
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Gria2^tm2Rsp/Gria2^+ Tg(Gria2*586N)238.2Rsp/0	involves: 129S1/Sv * 129X1/SvJ	MGI:3612464
cx2	Tg(Gria2*586N)238.2Rsp/0 Tg(SOD1*G93A)1Gur/0	involves: C57BL/6Ico	MGI:4834300
tg3	Tg(Gria2*586N)238.2Rsp/0	Not Specified	MGI:3612461

Genotype
MGI:3612464

cx1

• Allelic Composition: Gria2^tm2Rsp/Gria2⁺; Tg(Gria2*586N)238.2Rsp/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ

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

mortality/aging

premature death ( J:51845 )

• die before P25

nervous system

seizures ( J:51845 )

• exibit periodic spontaneous epileptic seizures that start at P14/15

abnormal hippocampus CA3 region morphology ( J:51845 )

• develop neurosclerosis in CA3 subfield

abnormal AMPA-mediated synaptic currents ( J:51845 )

• calcium permeability of AMPA receptors in CA1 pyramidal neurons is increased about 4-fold relative to wild-type

• significant increase in the macroscopic AMPA receptor conductance in nucleated patches of CA1 pyramidal cells

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:51845 )

• mutants are hyperexcitable

seizures ( J:51845 )

• exibit periodic spontaneous epileptic seizures that start at P14/15

Genotype
MGI:4834300

cx2

• Allelic Composition: Tg(Gria2*586N)238.2Rsp/0; Tg(SOD1*G93A)1Gur/0
• Genetic Background: involves: C57BL/6Ico

mortality/aging

premature death ( J:97818 )

• accelerated disease progression

• decreased survival (1932.93 vs. 208.45.06 days), compared with Tg(SOD1*G93A)1Gur mice

growth/size/body

weight loss ( J:97818 )

• declined body weight (14.3% over 51 days) after 20 weeks of age

behavior/neurological

impaired righting response ( J:97818 )

• lose righting reflex between 12 and 18 weeks

tremors ( J:97818 )

• accelerated onset (87.205.5 vs. 1856.3 days), compared with Tg(SOD1*G93A)1Gur mice

• in all 13 mice

Genotype
MGI:3612461

tg3

• Allelic Composition: Tg(Gria2*586N)238.2Rsp/0
• Genetic Background: Not Specified

growth/size/body

decreased body weight ( J:97818 )

• decreased body weight from 19 to 83 weeks

nervous system

abnormal ventromedial hypothalamic nucleus morphology ( J:97818 )

• neurons in different stages of degeneration and reactive gliosis in the ventromedial hypothalamic nucleus

• decreased density of neurons in the ventromedial hypothalamic nucleus of 12- and 24-month-old mice

abnormal amygdala morphology ( J:97818 )

• neurons in different stages of degeneration and reactive gliosis in the basolateral amygdaloid nucleus

• decreased density of neurons in the basolateral amygdaloid nucleus of 12- and 24-month-old mice

gliosis ( J:97818 )

• reactive gliosis in the ventral horns of lumbar spinal cord, basolateral amygdaloid nucleus, and ventromedial hypothalamic nucleus

motor neuron degeneration ( J:51845 , J:97818 )

• develop late motoneuron degeneration and motoneuron disease (J:51845)

• neurons in different stages of degeneration in the ventral horns of lumbar spinal cord, basolateral amygdaloid nucleus, and ventromedial hypothalamic nucleus (J:97818)

• vacuolar structures in the cytoplasm and darkening of the nucleoplasm and cytoplasm in degenerating neurons (J:97818)

• preservation of synaptic contacts onto degenerating neurons (J:97818)

abnormal spinal cord ventral horn morphology ( J:97818 )

• neurons in different stages of degeneration and reactive gliosis in the ventral horns of lumbar spinal cord

decreased spinal cord ventral horn cell number ( J:97818 )

• decreased density of neurons in the ventral horns of lumbar spinal cord of 12- and 24-month-old mice

abnormal AMPA-mediated synaptic currents ( J:51845 )

• 2-fold increase in the calcium permeability of AMPA receptors in nucleated CA1 cell patches

behavior/neurological

increased anxiety-related response ( J:97818 )

• anxiety between 19 and 75 weeks of age

• avoid the open arms of the maze, entering them less frequently and spending less time in them

• avoid punished crossings in the four-plate test

abnormal reflex ( J:97818 )

• plantar flexion first seen in 33% of the mice at 35 weeks vs 15-25% in wild-type mice at more advanced ages

• plantar flexions are progressively evident in 83% of the mice starting at 47 weeks, and persist up to the age of 83 weeks

• decreased spinal reflexes (H-reflexes and flexor reflexes) at 24 months

short stride length ( J:97818 )

• at aged 19 to 83 weeks

decreased locomotor activity ( J:97818 )

• decreased horizontal exploratory activity between 47 and 83 weeks

• move less total distance between 35 and 83 weeks

• lower average speed of movements between 63 and 83 weeks