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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Park2tm1Ccs
targeted mutation 1, Christine C Stichel
MGI:3764690
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Park2tm1Ccs/Park2tm1Ccs involves: 129S1/Sv * 129X1/SvJ * C57BL/6 MGI:3764702
hm2
Park2tm1Ccs/Park2tm1Ccs involves: 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:4939781
cx3
Park2tm1Ccs/Park2tm1Ccs
Tg(CAG-SNCA*)1.1Ccs/?
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 MGI:3764819
cx4
Park2tm1Ccs/Park2tm1Ccs
Tg(Th-SNCA*)1.2Ccs/?
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 MGI:3764820


Genotype
MGI:3764702
hm1
Allelic
Composition
Park2tm1Ccs/Park2tm1Ccs
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Park2tm1Ccs mutation (0 available); any Park2 mutation (20 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
N
• mice display normal motor skills
• unlike wild-type mice, exposure to amphetamine does not significantly affect behavior
• unlike wild-type mice, mice do not display increased exploration on the first day in a new environment, travel less horizontal distance, and exhibit increased thigmotaxic behavior
• mice exhibit a slightly impaired spatial learning ability in a Morris water maze test in which they made fewer crossings and did not selectively cross over the hidden platform
• mice exhibit increased anxiety behavior in a light dark transition test in which they spend less time in the illuminated compartment and make less transitions between light and dark compartments
• mice exhibit increased thigmotaxic behavior in an open field and water maze

nervous system
N
• mice exhibit normal brain morphology, and normal dopaminergic neurons, micro- and astrogliosis in the substantial nigra and locus coeruleus (J:127250)
• mice exhibit normal brain and spinal cord morphology, and normal dopaminergic neurons and axons, cell death or micro- and astrogliosis in the substantial nigra, striatum, locus coeruleus and cerebral cortex (J:127707)
• while dopamine uptake by cells is normal, monoamine metabolism of dopamine is increased in the striatum
• in old-aged mice, neurons display cytoplasmic vacuoles and disruptions in the Golgi network and endoplasmic reticulum
• in aged mice, neurons display cytoplasmic vacuoles, disruptions in the Golgi network and endoplasmic reticulum, occasionally detached outer nuclear membrane, and mitochondria abnormalities including electron dense inclusion bodies, dilated and disorganized cristae and protrusions

homeostasis/metabolism
• while dopamine uptake by cells is normal, monoamine metabolism of dopamine is increased in the striatum




Genotype
MGI:4939781
hm2
Allelic
Composition
Park2tm1Ccs/Park2tm1Ccs
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Park2tm1Ccs mutation (0 available); any Park2 mutation (20 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cellular
• almost all mesencephalic glial cell types exhibit a higher number of structurally altered mitochondria, showing disintegration and reduction of mitochondrial cristae, mitochondrial enlargement and formation of protrusions or disruption of the outer membrane
• mitochondrial damage is noticeable at 16 days of age in all mesencephalic cell types and increases 4-8 fold in the next few weeks
• mitochondrial alterations are higher in oligodendrocytes than in astrocytes or microglia

nervous system
• almost all mesencephalic glial cell types exhibit a higher number of structurally altered mitochondria
• mitochondrial alterations are higher in oligodendrocytes than in astrocytes or microglia
• mesencephalic glial cells exhibit a higher (2.5-7-fold) percentage of damaged mitochondria than in mesencephalic neurons at 3 and 12-15 months of age

Mouse Models of Human Disease
OMIM ID Ref(s)
Parkinson Disease 2, Autosomal Recessive Juvenile; PARK2 600116 J:168847




Genotype
MGI:3764819
cx3
Allelic
Composition
Park2tm1Ccs/Park2tm1Ccs
Tg(CAG-SNCA*)1.1Ccs/?
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Park2tm1Ccs mutation (0 available); any Park2 mutation (20 available)
Tg(CAG-SNCA*)1.1Ccs mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
N
• mice exhibit normal brain and spinal cord morphology, and normal dopaminergic neurons and axons, cell death or micro- and astrogliosis in the substantial nigra, striatum, locus coeruleus and cerebral cortex
• in old-aged mice, neurons display cytoplasmic vacuoles and disruptions in the Golgi network and endoplasmic reticulum
• in aged mice, neurons display cytoplasmic vacuoles, disruptions in the Golgi network and endoplasmic reticulum, occasionally detached outer nuclear membrane, and mitochondria abnormalities including electron dense inclusion bodies, dilated and disorganized cristae and protrusions

cellular
• the number of damaged mitochondria is increased 195% in the substantia nigra and up to 900% in the frontal cortex compared to in wild-type mice
• however, the number of mitochondria in affected brains is normal and no mitochondrial swelling is observed
• mitochondrial damage increases and the number of mitochondria decreases with age in the substantia nigra and frontal cortex
• respiration rate is decreased 29.5% when glutamate/malate is used as a substrate after treatment with CCCP
• state 3 respiration from complex I is reduced 18.7% compared to in wild-type mice
• however, respiratory rates starting at complex II or complex III/IV or state 4 are normal




Genotype
MGI:3764820
cx4
Allelic
Composition
Park2tm1Ccs/Park2tm1Ccs
Tg(Th-SNCA*)1.2Ccs/?
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Park2tm1Ccs mutation (0 available); any Park2 mutation (20 available)
Tg(Th-SNCA*)1.2Ccs mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
N
• mice exhibit normal brain and spinal cord morphology, and normal dopaminergic neurons and axons, cell death or micro- and astrogliosis in the substantial nigra, striatum, locus coeruleus and cerebral cortex
• in old-aged mice, neurons display cytoplasmic vacuoles and disruptions in the Golgi network and endoplasmic reticulum
• in aged mice, neurons display cytoplasmic vacuoles, disruptions in the Golgi network and endoplasmic reticulum, occasionally detached outer nuclear membrane, and mitochondria abnormalities including electron dense inclusion bodies, dilated and disorganized cristae and protrusions

cellular
• mitochondrial damage increases and the number of mitochondria decreases with age in the substantia nigra
• however, the number of mitochondria in affected brains is normal and no mitochondrial swelling is observed
• the number of damaged mitochondria in the substantia nigra is increased compared to in wild-type mice
• respiration rate is decreased 23.2% when glutamate/malate is used as a substrate after treatment with CCCP
• state 3 respiration from complex I is reduced 21.0% to 29.4% compared to in wild-type mice
• however, respiratory rates starting at complex II or complex III/IV or state 4 are normal





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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory