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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Atp1a2+
wild type
MGI:2430037
Summary 7 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
ht1
Atp1a2tm1.1Tmklh/Atp1a2+ B6.129S1(Cg)-Atp1a2tm1.1Tmklh MGI:6314222
ht2
Atp1a2tm2Kwk/Atp1a2+ B6.129X1-Atp1a2tm2Kwk MGI:3522672
ht3
Atp1a2tm1.1Gica/Atp1a2+ B6.Cg-Atp1a2tm1.1Gica MGI:6729923
ht4
Atp1a2tm2Ling/Atp1a2+ involves: 129P2/OlaHsd MGI:3694085
ht5
Atp1a2tm2Ling/Atp1a2+ involves: 129P2/OlaHsd * Black Swiss MGI:3697483
ht6
Atp1a2tm2Ling/Atp1a2+ involves: 129P2/OlaHsd * C57BL/6 * Black Swiss MGI:2451298
ht7
Atp1a2tm1Kwk/Atp1a2+ involves: 129X1/SvJ * C57BL/6J MGI:2669633


Genotype
MGI:6314222
ht1
Allelic
Composition
Atp1a2tm1.1Tmklh/Atp1a2+
Genetic
Background
B6.129S1(Cg)-Atp1a2tm1.1Tmklh
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp1a2tm1.1Tmklh mutation (0 available); any Atp1a2 mutation (60 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• females exhibit more distressed behaviors in the open field such as increased dropping and urination compared to wild-type mice
• mice show anhedonia in the sucrose-preference test when short- or long-lasting stress protocols are applied prior to the test
• however, unstressed mice exhibit normal sucrose-preference
• in the tail suspension test, mice exhibit a stress-induced depression-like phenotype, displaying increased immobility, reduced mobility, reduced movement, and fewer rotations than wild-type mice
• females display increased grooming compared to wild-type mice
• treatment of females with Depoprovera reduces the excessive grooming behavior
• females exhibit increased marble-burying behavior
• treatment of females with amantadine or memantine or Depoprovera rescues the increased marble-burying behavior
• however, mice exhibit normal behavior in the elevated plus maze indicting no anxiety
• both males and females show increased responsivity to aversive acoustic stimuli
• mice administered the weak NMDA type glutamate receptor antagonist amantadine show rescue of the increased acoustic startle reflex
• females spend less time on the accelerated rotarod indicating decreased endurance, learning and coordination
• however, grip strength is normal
• treatment of female mice with Depoprovera, a progestin-only contraceptive, rescues rotarod behaviors seen in females
• females exhibit hypolocomotion as they travel a shorter distance and move more slowly than wild-type females
• treatment of female mice with Depoprovera, a progestin-only contraceptive, rescues the open field behaviors seen in females
• in the three-chamber sociability test, mice show reduced sociability and spend comparable amounts of time in both chambers and comparable sniffing times to the stranger mouse and the empty cage and have the same number of entries into both chambers
• in the tail suspension test, about 50% of mice emit audible squeaks compared to about 14% of wild-type mice

growth/size/body
• males gain more weight over a period of 4 months than wild-type males
• however, females show normal body weight progression

homeostasis/metabolism
• females exhibit elevated glutamate levels in the cortex, hippocampus, and cerebellum
• treatment of females with Depoprovera reduces glutamate levels to wild-type levels
• mice show normal levels of dopamine, noradrenaline, serotonin and GABA in frontal and occipital cortex, striatum, hippocampus, brain stem, and cerebellum

nervous system
• recovery phase after cortical spreading depression is prolonged in males
• however, cerebral blood flow is similar to wild-type after cortical spreading depression induction

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
familial hemiplegic migraine 2 DOID:0111182 OMIM:602481
J:259556




Genotype
MGI:3522672
ht2
Allelic
Composition
Atp1a2tm2Kwk/Atp1a2+
Genetic
Background
B6.129X1-Atp1a2tm2Kwk
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp1a2tm2Kwk mutation (0 available); any Atp1a2 mutation (60 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• appeared normal and life-expectancy was comparable to wild-type




Genotype
MGI:6729923
ht3
Allelic
Composition
Atp1a2tm1.1Gica/Atp1a2+
Genetic
Background
B6.Cg-Atp1a2tm1.1Gica
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp1a2tm1.1Gica mutation (0 available); any Atp1a2 mutation (60 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• mice show increased susceptibility to cortical spreading depression, with a decreased induction of threshold and an increased velocity of propagation of cortical spreading depression
• however, duration of cortical spreading depression and induction of repetitive cortical spreading depression are not affected

behavior/neurological
• transfer arousal testing indicates increased anxiety and fear
• however, mice show no major differences in any other sensory-motor functions
• mice exhibit increased anxiety
• mice exhibit increased fear

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
familial hemiplegic migraine 2 DOID:0111182 OMIM:602481
J:307518




Genotype
MGI:3694085
ht4
Allelic
Composition
Atp1a2tm2Ling/Atp1a2+
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp1a2tm2Ling mutation (0 available); any Atp1a2 mutation (60 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
muscle
• in isometric measurements of isolated EDL muscle, more force is produced at any given voltage, and has higher force is at supramaximal voltage compared to wild-type; peak tetanic force is 384 mN/sq. mm compared to 297 mN/sq. mm in wild-type
• rate of force development in tetanic contraction is significantly increased compared to wild-type
• EDL twitch force is 34% higher than that produced by wild-type
• constant for fatigue is similar to wild-type




Genotype
MGI:3697483
ht5
Allelic
Composition
Atp1a2tm2Ling/Atp1a2+
Genetic
Background
involves: 129P2/OlaHsd * Black Swiss
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp1a2tm2Ling mutation (0 available); any Atp1a2 mutation (60 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• increased latency to find the platform in a Morris water maze
• fewer entries into the open arms and less time spent in the open quadrants in an elevated zero maze
• less time spent in the center and more time spent in the corners of an open field and reduced movement in the first time period after being placed in an open field

homeostasis/metabolism
N
• circulating levels of corticosterone, norepinephrine, dopamine, serotonin, and their metabolites are all similar to wild-type mice




Genotype
MGI:2451298
ht6
Allelic
Composition
Atp1a2tm2Ling/Atp1a2+
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * Black Swiss
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp1a2tm2Ling mutation (0 available); any Atp1a2 mutation (60 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• hearts are hypercontractile

muscle
• hearts are hypercontractile




Genotype
MGI:2669633
ht7
Allelic
Composition
Atp1a2tm1Kwk/Atp1a2+
Genetic
Background
involves: 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp1a2tm1Kwk mutation (0 available); any Atp1a2 mutation (60 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• heterozygous mutant mice show increased fear/anxiety behaviors in a variety of tests
• after conditioned fear stimuli, c-fos expression is higher in the amygdala and piriform cortex than in wild-type and unstimulated heterozygous mice





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last database update
03/18/2025
MGI 6.24
The Jackson Laboratory