Journal of Biological Chemistry · 2003 · 152 citations · 37 references
Life SciencesMolecular BiologyNeuroscienceMolecular NeurobiologyMolecular NeuroscienceNeurodegenerationMolecular Basis Of NeurodegenerationNeurodegenerative DiseasesNeurodegenerative DisordersNeuropathology Of AgingParkinson DiseaseProtein DegradationAlpha-synuclein MisfoldingAntibodiesDopaminergic NeuronsProteasomal TurnoverProtein ExtractionUbiquitin BindingUbiquitin SystemUbiquitin-proteasome System Dysfunction
Autosomal recessive juvenile parkinsonism is a movement disorder associated with the degeneration of dopaminergic neurons in substantia nigra pars compacta. The loss of functional parkin caused by parkin gene mutations is the most common single cause of juvenile parkinsonism. Parkin has been shown to aid in protecting cells from endoplasmic reticulum and oxidative stressors presumably due to ubiquitin ligase activity of parkin that targets proteins for proteasomal degradation. However, studies on parkin have been impeded because of limited reagents specific for this protein. Here we report the generation and characterization of a panel of parkin-specific monoclonal antibodies. Biochemical analyses indicate that parkin is present only in the high salt-extractable fraction of mouse brain, whereas it is present in both the high salt-extractable and RIPA-resistant, SDS-extractable fraction in young human brain. Parkin is present at decreased levels in the high salt-extractable fraction and at increased levels in the SDS-extractable fraction from aged human brain. This shift in the extractability of parkin upon aging is seen in humans but not in mice, demonstrating species-specific differences in the biochemical characteristics of murine versus human parkin. Finally, by using these highly specific anti-parkin monoclonal antibodies, it was not possible to detect parkin in alpha-synuclein-containing lesions in alpha-synucleinopathies, thereby challenging prior inferences about the role of parkin in movement disorders other than autosomal recessive juvenile parkinsonism.
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Initial sequencing and analysis of the human genome
Eric S. Lander, Lauren Linton, Bruce W. Birren et al. · Nature · 2001 · 24.6K citations · Full text
Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism
Tohru Kitada, Shuichi Asakawa, Nobutaka Hattori et al. · Nature · 1998 · 5.2K citations