Phenotypes associated with this allele

• Allele Symbol: Mt3^tm1Rpa
• Allele Name: targeted mutation 1, Richard D Palmiter
• Allele ID: MGI:2389488
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Mt3^tm1Rpa/Mt3^tm1Rpa	involves: 129S7/SvEvBrd * C57BL/6	MGI:3574994
cx2	Mt3^tm1Rpa/Mt3^tm1Rpa Tg(SOD1*G93A)^dl1Gur/0	involves: 129S7/SvEvBrd * C57BL/6 * SJL	MGI:3574995

Genotype
MGI:3574994

hm1

• Allelic Composition: Mt3^tm1Rpa/Mt3^tm1Rpa
• Genetic Background: involves: 129S7/SvEvBrd * C57BL/6

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

behavior/neurological

increased susceptibility to pharmacologically induced seizures ( J:38222 )

• susceptibility and severity of seizures induced by Kainic acid was increased

• a higher percentage of mice exhibited convulsions and the latency to seizure was shorter

• Increased CA3 neuron damage as a result of seizures

homeostasis/metabolism

abnormal mineral level ( J:38222 )

• zinc deficiency in the brain of about 12%

• levels of other minerals are normal

nervous system

increased susceptibility to pharmacologically induced seizures ( J:38222 )

• susceptibility and severity of seizures induced by Kainic acid was increased

• a higher percentage of mice exhibited convulsions and the latency to seizure was shorter

• Increased CA3 neuron damage as a result of seizures

abnormal thalamus morphology ( J:86693 )

• zinc does not accumulate in cell bodies of the thalamus after seizures as it does in control mice

abnormal hippocampus morphology ( J:86693 )

abnormal Ammon gyrus morphology ( J:86693 )

• zinc accumulates in neurons of CA1 and CA3 after seizures

• high level of cell death in CA3 neurons but CA1 neurons are relatively protected

abnormal hippocampal mossy fiber morphology ( J:86693 )

• zinc lost from mossy fiber terminals after kainic acid induced seizures

abnormal astrocyte morphology ( J:38222 )

• increased numbers of astrocytes apparently present in 2 year old mice as compared to age matched controls

Genotype
MGI:3574995

cx2

• Allelic Composition: Mt3^tm1Rpa/Mt3^tm1Rpa; Tg(SOD1*G93A)^dl1Gur/0
• Genetic Background: involves: 129S7/SvEvBrd * C57BL/6 * SJL

mortality/aging

premature death ( J:79760 )

• markedly reduced survival, by about 20%

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:79760 )

• severe declines in motor function starting around 5-6 months of age

decreased grip strength ( J:79760 )

• reduced

nervous system

decreased motor neuron number ( J:79760 )

• becomes severe after mice begin to display behavioral symptoms