Phenotypes associated with this allele

• Allele Symbol: Kcnn2^fri
• Allele Name: frissonant
• Allele ID: MGI:1856876
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Kcnn2^fri/Kcnn2^fri	129S2.C3Pas-Kcnn2^fri	MGI:5296000
hm2	Kcnn2^fri/Kcnn2^fri	involves: C3H	MGI:3765154

Genotype
MGI:5296000

hm1

• Allelic Composition: Kcnn2^fri/Kcnn2^fri
• Genetic Background: 129S2.C3Pas-Kcnn2^fri

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

reproductive system

reproductive system phenotype ( J:177761 )

N • male mice exhibit normal spermatozoid counts and motility

N • female mice exhibit normal reproduction

reduced male fertility ( J:177761 )

♂

nervous system

abnormal afterhyperpolarization ( J:177761 )

• MVN neurons fail to exhibit double-component afterhyperpolarizations (AHPs)

• all MVN neurons are classified as type A neurons unlike wild-type neurons include types A, B, and C

• no MVN neurons exhibit double AHP unlike in wild-type neurons

• AHPR is increased compared to in wild-type neurons

• latency of AHPR is reduced compared to in wild-type neurons

abnormal single cell response ( J:177761 )

• 4 of 29 (14%) medial vestibular nucleus (MVN) neurons are silent compared to 5% of wild-type neurons

• 17% of MVN neurons exhibit regular oscillation of the baseline field potential unlike in wild-type neurons

• MVN neurons exhibit increased concavity and decreased convexity compared to in wild-type neurons

• apamin has no effect on the membrane and firing properties of MVN neurons

• however, MVN neurons exhibit normal spontaneous firing rate and regularity of the discharge

behavior/neurological

tremors ( J:177761 )

• constant and rapid tremor as early as 6 days of age

homeostasis/metabolism

decreased physiological sensitivity to xenobiotic ( J:177761 )

• apamin has no effect on the membrane and firing properties of medial vestibular nucleus (MVN) neurons unlike wild-type neurons

growth/size/body

decreased body size ( J:177761 )

• with the difference slowly attenuating after weaning

Genotype
MGI:3765154

hm2

• Allelic Composition: Kcnn2^fri/Kcnn2^fri
• Genetic Background: involves: C3H

behavior/neurological

abnormal short-term object recognition memory ( J:70213 )

tremors ( J:13711 , J:70213 )

• rapid tremors reminiscent of the quaking phenotype (J:13711)

• tremors are Parkinson-like (J:70213)

abnormal motor coordination/balance ( J:70213 )

• 10,20,and 40 mg/kg L-Dopa improved motor performance

• 10 mg/kg Seligiline treatments improved motor performance

• 20 mg/kg Ropinirole treatment increased motor performance

impaired limb coordination ( J:70213 )

• unable to walk on a rotarod bar

• unable to normally traverse an inclined plane

• while suspended unable to coordinate hindpaws to grasp thread held by front paws

abnormal gait ( J:70213 )

• width of foot placement is greater than for control

short stride length ( J:70213 )

• intrastep distance is also shorter

decreased vertical activity ( J:70213 )

• number rearings significantly increased following adminstration of between 10 and 60 mg/kg L-Dopa

nervous system

abnormal brain white matter morphology ( J:13711 )

• histological evidence of degeneration

abnormal spinal cord white matter morphology ( J:13711 )

• histological evidence of degeneration

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Parkinson's disease		DOID:14330	OMIM:PS168600	J:70213