Mouse Genome Informatics
cx1
    Park2tm1Ccs/Park2tm1Ccs
Tg(Th-SNCA*)1.2Ccs/?

involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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 (J:127707)
• 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


Mouse Genome Informatics
tg2
    Tg(Th-SNCA*)1.2Ccs/?
involves: C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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 substantia nigra, striatum, locus coeruleus and cerebral cortex (J:127707)
• mice exhibit normal susbstantia nigra compacta (J:127708)
• in old-aged mice, neurons display cytoplasmic vacuoles and disruptions in the Golgi network and endoplasmic reticulum
• 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
• 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
• astrocytes exhibit reduced mitochondrial calcium storage capacity

cellular
• however, the number of mitochondria in affected brains is normal and no mitochondrial swelling is observed (J:127707)
• the number of damaged mitochondria in the substantia nigra is increased 340% compared to in wild-type mice (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)
• astrocytes exhibit reduced mitochondrial calcium storage capacity

Mouse Models of Human Disease
OMIM IDRef(s)
Parkinson Disease, Late-Onset; PD 168600 J:168847