B6.Cg-Prkntm1Ccs/Ieg
| Status | Available to order |
| EMMA ID | EM:14605 |
| International strain name | B6.Cg-Prkntm1Ccs/Ieg |
| Alternative name | B6.Cg-Park2tm1Hlu |
| Strain type | Targeted Mutant Strains : Knock-out |
| Allele/Transgene symbol | Prkntm1Ccs, |
| Gene/Transgene symbol | Prkn |
Information from provider
| Provider | Xinran Zhu |
| Provider affiliation | Lehrstuhl für Tierphysiologie, Ruhr-Universität Bochum |
| Genetic information | A targeting construct in which the Parkin exons 3, was replaced with a neomycin (Neo) selectable cassette flanked with two loxP elements. The neomycin (Neo) selectable cassette was subsequently deleted by crossing with a cre deleter strain. RT-PCR shows that exon 2 splices to exon 4, skipping exon 3 entirely, resulting in a frame shift and a premature stop codon in exon 5. |
| Phenotypic information | Homozygous:Parkin-knockout mice (PaKO) were viable, fertile, and showed no gross anatomical or neuronal abnormalities. Dopaminergic neuron (DA) numbers in the substantia nigra and fiber densities and dopamine levels in the striatum were normal. PaKO did not enhance vulnerability of dopaminergic neurons to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) toxicity. However, they displayed impaired exploration and habituation to a new environment and exhibited thigmotaxis behaviour in the open field and Morris water maze. Abnormal anxiety-related behaviour of PaKO was also observed in the light/dark exploration test paradigm. Dopamine metabolism was enhanced in the striatum of mutant mice, as revealed by increased homovanillic acid (HVA) content and a reduced ratio of dihydroxyphenylacetic acid (DOPAC) ⁄HVA. The alterations found in the dopaminergic system could be responsible for the behavioural impairments of PaKO mice. Accumulation of damaged mitochondrion were observed in DA neurons and in glia cells within the region as well, suggesting abnormal mitophagy in Da system of PaKO mice.Heterozygous:It has not been investigated so far |
| Breeding history | Backcrossed to C57BL/6 for 20 generations |
| References |
|
| Homozygous fertile | yes |
| Homozygous viable | yes |
| Homozygous matings required | no |
| Immunocompromised | not known |
Information from EMMA
| Archiving centre | Helmholtz Zentrum Muenchen - German Research Center for Environmental Health (GmbH), Oberschleißheim, Germany |
| Animals used for archiving | homozygous C57BL/6 |
Disease and phenotype information
Orphanet associated rare diseases, based on orthologous gene matching
- Young-onset Parkinson disease / Orphanet_2828
Literature references
- Mono- and double-mutant mouse models of Parkinson's disease display severe mitochondrial damage.;Stichel Christine C, Zhu Xin-Ran, Bader Verian, Linnartz Bettina, Schmidt Saskia, Lübbert Hermann, ;2007;Human molecular genetics;16;2377-93; 17412759
- Genetic mouse models for Parkinson's disease display severe pathology in glial cell mitochondria.;Schmidt Saskia, Linnartz Bettina, Mendritzki Sonja, Sczepan Teresa, Lübbert Matthias, Stichel Christine C, Lübbert Hermann, ;2011;Human molecular genetics;20;1197-211; 21212098
- Non-motor behavioural impairments in parkin-deficient mice.;Zhu Xin-Ran, Maskri Lyutha, Herold Christina, Bader Verian, Stichel Christine C, Güntürkün Onur, Lübbert Hermann, ;2007;The European journal of neuroscience;26;1902-11; 17883413