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PMID: 15684050 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't

Parkin-deficient mice are not a robust model of parkinsonism.

Perez FA, Palmiter RD

Abstract

Mutations in the human parkin gene cause autosomal recessive juvenile parkinsonism, a heritable form of Parkinson's disease (PD). To determine whether mutations in the mouse parkin gene (Park2) also result in a parkinsonian phenotype, we generated mice with a targeted deletion of parkin exon 2. Using an extensive behavioral screen, we evaluated neurological function, motor ability, emotionality, learning, and memory in aged Parkin-deficient mice. The behavioral profile of Parkin-deficient mice on a B6;129S4 genetic background was strikingly similar to that of control mice, and most differences were not reproducible by using coisogenic mice on a 129S4 genetic background. Moreover, catecholamine levels in the striatum, olfactory bulb, and spinal cord of Parkin-deficient mice were normal. In contrast to previous studies using independently generated Parkin-deficient mice, we found no evidence for nigrostriatal, cognitive, or noradrenergic dysfunction. Understanding why Parkin-deficient mice do not exhibit robust signs of parkinsonism could advance knowledge and treatment of PD.

MeSH Terms
Amphetamines/pharmacology Animals Behavior, Animal/physiology Disease Models, Animal Emotions Genotype Humans Learning Memory Mice Mice, Inbred C57BL Mice, Knockout Motor Activity/drug effects,physiology Nerve Tissue/chemistry Parkinson Disease/genetics,metabolism,physiopathology Phenotype Ubiquitin-Protein Ligases/genetics,metabolism
Chemicals
Amphetamines Ubiquitin-Protein Ligases parkin protein
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Perez Francisco A
Graduate Program in Neurobiology and Behavior, University of Washington, Seattle, WA 98195, USA.
Palmiter Richard D
References (42)
42 references, click to expand
  1. Mitochondrial dysfunction and oxidative damage in parkin-deficient mice.
    J Biol Chem. 2004 Apr 30;279(18):18614-22 PMID: 14985362
  2. A new continuous alternation task in T-maze detects hippocampal dysfunction in mice. A strain comparison and lesion study.
    Behav Brain Res. 1998 Sep;95(1):91-101 PMID: 9754881
  3. parkin mutation analysis in clinic patients with early-onset Parkinson [corrected] disease.
    Am J Med Genet A. 2004 Aug 15;129A(1):44-50 PMID: 15266615
  4. Loss of locus coeruleus neurons and reduced startle in parkin null mice.
    Proc Natl Acad Sci U S A. 2004 Jul 20;101(29):10744-9 PMID: 15249681
  5. Electrophysiological characterisation of the dentate gyrus in five inbred strains of mouse.
    Brain Res. 1999 Sep 11;841(1-2):123-34 PMID: 10546995
  6. Parkin functions as an E2-dependent ubiquitin- protein ligase and promotes the degradation of the synaptic vesicle-associated protein, CDCrel-1.
    Proc Natl Acad Sci U S A. 2000 Nov 21;97(24):13354-9 PMID: 11078524
  7. Parkin suppresses unfolded protein stress-induced cell death through its E3 ubiquitin-protein ligase activity.
    J Biol Chem. 2000 Nov 17;275(46):35661-4 PMID: 10973942
  8. Origin of the mutations in the parkin gene in Europe: exon rearrangements are independent recurrent events, whereas point mutations may result from Founder effects.
    Am J Hum Genet. 2001 Mar;68(3):617-26 PMID: 11179010
  9. Strain-specific effects of amphetamine on prepulse inhibition and patterns of locomotor behavior in mice.
    J Pharmacol Exp Ther. 2001 Jul;298(1):148-55 PMID: 11408536
  10. Mapping of quantitative trait loci with knockout/congenic strains.
    Genome Res. 2001 Sep;11(9):1549-52 PMID: 11544198
  11. SEE: a tool for the visualization and analysis of rodent exploratory behavior.
    Neurosci Biobehav Rev. 2001 Jul;25(5):409-26 PMID: 11566479
  12. Gene targeting: technical confounds and potential solutions in behavioral brain research.
    Behav Brain Res. 2001 Nov 1;125(1-2):13-21 PMID: 11682088
  13. Controlling the false discovery rate in behavior genetics research.
    Behav Brain Res. 2001 Nov 1;125(1-2):279-84 PMID: 11682119
  14. Abnormal postnatal ontogeny of the locus coeruleus in the epileptic mutant mouse quaking.
    Brain Res Dev Brain Res. 2002 Jun 30;136(2):197-205 PMID: 12101037
  15. Complex relationship between Parkin mutations and Parkinson disease.
    Am J Med Genet. 2002 Jul 8;114(5):584-91 PMID: 12116199
  16. Targeted insertion results in a rhombomere 2-specific Hoxa2 knockdown and ectopic activation of Hoxa1 expression.
    Dev Dyn. 2002 Nov;225(3):305-15 PMID: 12412013
  17. Parkin binds the Rpn10 subunit of 26S proteasomes through its ubiquitin-like domain.
    EMBO Rep. 2003 Mar;4(3):301-6 PMID: 12634850
  18. The cellular protein level of parkin is regulated by its ubiquitin-like domain.
    J Biol Chem. 2003 May 2;278(18):16054-8 PMID: 12621021
  19. Parkin disease: a phenotypic study of a large case series.
    Brain. 2003 Jun;126(Pt 6):1279-92 PMID: 12764051
  20. A potential proteasome-interacting motif within the ubiquitin-like domain of parkin and other proteins.
    Trends Biochem Sci. 2003 Jun;28(6):280-3 PMID: 12826399
  21. How much phenotypic variation can be attributed to parkin genotype?
    Ann Neurol. 2003 Aug;54(2):176-85 PMID: 12891670
  22. Parkin gene inactivation alters behaviour and dopamine neurotransmission in the mouse.
    Hum Mol Genet. 2003 Sep 15;12(18):2277-91 PMID: 12915482
  23. Parkin-deficient mice exhibit nigrostriatal deficits but not loss of dopaminergic neurons.
    J Biol Chem. 2003 Oct 31;278(44):43628-35 PMID: 12930822
  24. Novel monoclonal antibodies demonstrate biochemical variation of brain parkin with age.
    J Biol Chem. 2003 Nov 28;278(48):48120-8 PMID: 12972409
  25. Spontaneous hypomorphic mutations in antioxidant enzymes of mice.
    Free Radic Biol Med. 2003 Dec 15;35(12):1645-52 PMID: 14680687
  26. It's a double knock-out! The quaking mouse is a spontaneous deletion of parkin and parkin co-regulated gene (PACRG).
    Mov Disord. 2004 Jan;19(1):101-4 PMID: 14743368
  27. The neurological mutant quaking(viable) is Parkin deficient.
    Mamm Genome. 2004 Mar;15(3):210-7 PMID: 15014970
  28. Parkin expression in the developing mouse.
    Brain Res Dev Brain Res. 2004 Apr 19;149(2):131-42 PMID: 15063093
  29. Parkin transcript variants in rat and human brain.
    Neurochem Res. 2004 Sep;29(9):1715-24 PMID: 15453267
  30. The locus coeruleus: a quantitative and genetic study in mice.
    Brain Res. 1982 Nov 4;250(2):353-7 PMID: 7171993
  31. Gene-targeting studies of mammalian behavior: is it the mutation or the background genotype?
    Trends Neurosci. 1996 May;19(5):177-81 PMID: 8723200
  32. Epidemiology of Parkinson's disease.
    Neurol Clin. 1996 May;14(2):317-35 PMID: 8827174
  33. Long-range disruption of gene expression by a selectable marker cassette.
    Proc Natl Acad Sci U S A. 1996 Nov 12;93(23):13090-5 PMID: 8917549
  34. Genetic variation among 129 substrains and its importance for targeted mutagenesis in mice.
    Nat Genet. 1997 May;16(1):19-27 PMID: 9140391
  35. Genealogy of the 129 inbred strains: 129/SvJ is a contaminated inbred strain.
    Mamm Genome. 1997 Jun;8(6):390-3 PMID: 9166580
  36. Inbred strain differences in prepulse inhibition of the mouse startle response.
    Psychopharmacology (Berl). 1997 Jul;132(2):169-80 PMID: 9266614
  37. Behavioral and functional analysis of mouse phenotype: SHIRPA, a proposed protocol for comprehensive phenotype assessment.
    Mamm Genome. 1997 Oct;8(10):711-3 PMID: 9321461
  38. Mutant mice and neuroscience: recommendations concerning genetic background. Banbury Conference on genetic background in mice.
    Neuron. 1997 Oct;19(4):755-9 PMID: 9354323
  39. Assessing the effects of the 129/Sv genetic background on swimming navigation learning in transgenic mutants: a study using mice with a modified beta-amyloid precursor protein gene.
    Brain Res. 1997 Oct 10;771(1):1-13 PMID: 9383002
  40. Locomotor activity in D2 dopamine receptor-deficient mice is determined by gene dosage, genetic background, and developmental adaptations.
    J Neurosci. 1998 May 1;18(9):3470-9 PMID: 9547254
  41. Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism.
    Nature. 1998 Apr 9;392(6676):605-8 PMID: 9560156
  42. Psychiatric aspects of Parkinson's disease--an update.
    J Neurol. 2004 Jul;251(7):795-804 PMID: 15258780
Article Info
Journal
Proceedings of the National Academy of Sciences of the United States of America
Abbr.
Proc Natl Acad Sci U S A
ISSN
0027-8424
Published
2005-02-08
Epub
2005-00-31
Pages
2174-9
Language
English
Region
United States
NLM ID
7505876
PMCID
PMC548311
Subset
IM
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