Phenotypes associated with this allele

• Allele Symbol: Tg(CAG-SNCA*)1.1Ccs
• Allele Name: transgene insertion 1.1, Christine C Stichel
• Allele ID: MGI:3764813
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Prkn^tm1Ccs/Prkn^tm1Ccs Tg(CAG-SNCA*)1.1Ccs/?	involves: 129S1/Sv * 129X1/SvJ * C57BL/6	MGI:3764819
tg2	Tg(CAG-SNCA*)1.1Ccs/?	involves: C57BL/6	MGI:3764817

Genotype
MGI:3764819

cx1

• Allelic Composition: Prkn^tm1Ccs/Prkn^tm1Ccs; Tg(CAG-SNCA*)1.1Ccs/?
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6

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

nervous system

nervous system phenotype ( J:127707 )

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

brain vacuoles ( J:127707 )

• in old-aged mice, neurons display cytoplasmic vacuoles and disruptions in the Golgi network and endoplasmic reticulum

abnormal neuron morphology ( J:127707 )

• 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

abnormal mitochondrial morphology ( J:127707 )

• 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

abnormal cellular respiration ( J:127707 )

• respiration rate is decreased 29.5% when glutamate/malate is used as a substrate after treatment with CCCP

abnormal mitochondrial ATP synthesis coupled electron transport ( J:127707 )

• 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:3764817

tg2

• Allelic Composition: Tg(CAG-SNCA*)1.1Ccs/?
• Genetic Background: involves: C57BL/6

nervous system

nervous system phenotype ( J:127708 , J:127707 )

N • mice exhibit normal susbstantia nigra compacta (J:127708)

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 (J:127707)

brain vacuoles ( J:127707 )

• in old-aged mice, neurons display cytoplasmic vacuoles and disruptions in the Golgi network and endoplasmic reticulum

abnormal CNS glial cell morphology ( J:168847 )

• 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 percentage of damaged mitochondria than in mesencephalic neurons at 3 and 12-15 months of age

abnormal neuron morphology ( J:127707 )

• 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

abnormal astrocyte physiology ( J:168847 )

• astrocytes exhibit reduced mitochondrial calcium storage capacity

cellular

abnormal mitochondrial morphology ( J:127707 , J:168847 )

• the number of damaged mitochondria is increased in the substantia nigra and up to 900% in the frontal cortex compared to in wild-type mice (J:127707)

• however, the number of mitochondria in affected brains is normal and no mitochondrial swelling is observed (J:127707)

• 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 (J:168847)

abnormal mitochondrial physiology ( J:168847 )

• astrocytes exhibit reduced mitochondrial calcium storage capacity

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Parkinson's disease		DOID:14330	OMIM:PS168600	J:168847