Mechanisms and models of α-Synuclein-related neurodegeneration

Wolfdieter Springer, Philipp J. Kable

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Expression of the Parkinson's disease-associated protein α-synuclein causes formation of aggregates and cytotoxicity in a great diversity of transgenic model organisms, in the case of Drosophila melanogaster affecting specific dopaminergic neuron clusters. The relative contribution of α-synuclein misfolding and phosphorylation for neurodegeneration was elucidated in these systems. In transgenic mice, typical neuropathologic inclusions formed concomitant with behavioral deficits, reminiscent of Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy. Neuronal degeneration was cell-autonomous in the Lewy body disease models, whereas gliotic changes accompanied neurodegeneration caused by (oligodendro)glial cytoplasmic inclusions. These recent findings provided major insights into the molecular mechanisms of α-synucleinopathies.

Original languageEnglish (US)
Pages (from-to)432-436
Number of pages5
JournalCurrent Neurology and Neuroscience Reports
Volume6
Issue number5
DOIs
StatePublished - Sep 2006
Externally publishedYes

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Synucleins
Lewy Body Disease
Genetically Modified Organisms
Multiple System Atrophy
Dopaminergic Neurons
Inclusion Bodies
Drosophila melanogaster
Neuroglia
Transgenic Mice
Parkinson Disease
Phosphorylation

ASJC Scopus subject areas

  • Medicine(all)
  • Neuroscience(all)

Cite this

Mechanisms and models of α-Synuclein-related neurodegeneration. / Springer, Wolfdieter; Kable, Philipp J.

In: Current Neurology and Neuroscience Reports, Vol. 6, No. 5, 09.2006, p. 432-436.

Research output: Contribution to journalArticle

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