All Phenotypes Kcnq3<tm1.1Naas> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Kcnq3^tm1.1Naas
targeted mutation 1.1, Nanda A Singh
MGI:4397593

Summary

6 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Kcnq3^tm1.1Naas/Kcnq3^tm1.1Naas	B6.129-Kcnq3^tm1.1Naas	MGI:4397675
hm2	Kcnq3^tm1.1Naas/Kcnq3^tm1.1Naas	FVB.129-Kcnq3^tm1.1Naas	MGI:4397677
hm3	Kcnq3^tm1.1Naas/Kcnq3^tm1.1Naas	involves: 129S1/Sv * 129X1/SvJ * C57BL/6	MGI:4397673
hm4	Kcnq3^tm1.1Naas/Kcnq3^tm1.1Naas	involves: 129S1/Sv * 129X1/SvJ * FVB/N	MGI:4397676
ht5	Kcnq3^tm1.1Naas/Kcnq3⁺	involves: 129S1/Sv * 129X1/SvJ * C57BL/6	MGI:4397674
ht6	Kcnq3^tm1.1Naas/Kcnq3⁺	involves: 129S1/Sv * 129X1/SvJ * FVB/N	MGI:4397678

Allelic Composition	Kcnq3^tm1.1Naas/Kcnq3^tm1.1Naas
Genetic Background	B6.129-Kcnq3^tm1.1Naas

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kcnq3^tm1.1Naas mutation (0 available); any Kcnq3 mutation (48 available)

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

mortality/aging

prenatal lethality, incomplete penetrance ( J:154582 )

• fewer than expected mice were born

nervous system

seizures ( J:154582 )

• mice exhibit rare spontaneous seizures

• 50% of mice exhibit a single severe seizures between weaning and P72

growth/size/body

decreased body size ( J:154582 )

behavior/neurological

seizures ( J:154582 )

• mice exhibit rare spontaneous seizures

• 50% of mice exhibit a single severe seizures between weaning and P72

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
benign neonatal seizures		DOID:14264	OMIM:121200 OMIM:121201 OMIM:269720	J:154582

Allelic Composition	Kcnq3^tm1.1Naas/Kcnq3^tm1.1Naas
Genetic Background	FVB.129-Kcnq3^tm1.1Naas

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kcnq3^tm1.1Naas mutation (0 available); any Kcnq3 mutation (48 available)

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

mortality/aging

premature death ( J:154582 )

• after P15, mice exhibit increased mortality compared with wild-type mice

• 20% of mice die during or after a spontaneous seizure

neonatal lethality, incomplete penetrance ( J:154582 )

• some mice die at P0

nervous system

nervous system phenotype ( J:154582 )

N	• despite seizures, mice exhibit no hippocampal mossy fiber sprouting or neuronal loss

seizures ( J:154582 )

• mice exhibit spontaneous seizures as early as the second postnatal week unlike wild-type mice

• after weaning, mice exhibit multiple spontaneous recurrent seizures unlike wild-type mice

• at P30 to P45, mice exhibit a decline in seizure severity

• by P65 to P75, mice exhibit low grade seizures with bilateral forelimb clonus, orofacial automatisms, jaw chomping, head clonus, occasional falling, and rearing

• clusters of seizures increase with age while severity decreases

abnormal hippocampus morphology ( J:154582 )

• after recurrent seizures, mice exhibit selected regions of necrosis in the hippocampus unlike in wild-type mice

astrocytosis ( J:154582 )

• in multiple hippocampal regions at P32 and P120

growth/size/body

decreased body size ( J:154582 )

behavior/neurological

seizures ( J:154582 )

• clusters of seizures increase with age while severity decreases

• mice exhibit spontaneous seizures as early as the second postnatal week unlike wild-type mice

• after weaning, mice exhibit multiple spontaneous recurrent seizures unlike wild-type mice

• at P30 to P45, mice exhibit a decline in seizure severity

• by P65 to P75, mice exhibit low grade seizures with bilateral forelimb clonus, orofacial automatisms, jaw chomping, head clonus, occasional falling, and rearing

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
benign neonatal seizures		DOID:14264	OMIM:121200 OMIM:121201 OMIM:269720	J:154582

Allelic Composition	Kcnq3^tm1.1Naas/Kcnq3^tm1.1Naas
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kcnq3^tm1.1Naas mutation (0 available); any Kcnq3 mutation (48 available)

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

mortality/aging

prenatal lethality, incomplete penetrance ( J:154582 )

• fewer than expected mice were born

nervous system

seizures ( J:154582 )

• mice exhibit spontaneous seizures with frequent generalized interictal cortical discharges, forepaw grooming, and clonic twitching unlike wild-type mice

abnormal neuron physiology ( J:154582 )

• CA1 neurons exhibit decreased M-type potassium ion current amplitude and density compared with wild-type neurons

behavior/neurological

seizures ( J:154582 )

• mice exhibit spontaneous seizures with frequent generalized interictal cortical discharges, forepaw grooming, and clonic twitching unlike wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
benign neonatal seizures		DOID:14264	OMIM:121200 OMIM:121201 OMIM:269720	J:154582

Allelic Composition	Kcnq3^tm1.1Naas/Kcnq3^tm1.1Naas
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kcnq3^tm1.1Naas mutation (0 available); any Kcnq3 mutation (48 available)

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

mortality/aging

prenatal lethality, incomplete penetrance ( J:154582 )

• fewer than expected mice were born

nervous system

abnormal neuron physiology ( J:154582 )

• CA1 neurons exhibit decreased M-type potassium ion current amplitude and density compared with wild-type neurons

Allelic Composition	Kcnq3^tm1.1Naas/Kcnq3⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kcnq3^tm1.1Naas mutation (0 available); any Kcnq3 mutation (48 available)

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

behavior/neurological

abnormal seizure response to electrical stimulation ( J:154582 )

• mice exhibit decreased threshold to convulsive current at which 50% of mice exhibit tonic hindlimb extension seizure compared with wild-type mice

nervous system

abnormal seizure response to electrical stimulation ( J:154582 )

• mice exhibit decreased threshold to convulsive current at which 50% of mice exhibit tonic hindlimb extension seizure compared with wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
benign neonatal seizures		DOID:14264	OMIM:121200 OMIM:121201 OMIM:269720	J:154582

Allelic Composition	Kcnq3^tm1.1Naas/Kcnq3⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kcnq3^tm1.1Naas mutation (0 available); any Kcnq3 mutation (48 available)

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

behavior/neurological

abnormal seizure response to electrical stimulation ( J:154582 )

• mice exhibit decreased threshold to convulsive current at which 50% of mice exhibit tonic hindlimb extension seizure compared with wild-type mice

nervous system

abnormal seizure response to electrical stimulation ( J:154582 )

• mice exhibit decreased threshold to convulsive current at which 50% of mice exhibit tonic hindlimb extension seizure compared with wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
benign neonatal seizures		DOID:14264	OMIM:121200 OMIM:121201 OMIM:269720	J:154582

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