RGD Reference Report - Gamma-synucleinopathy: neurodegeneration associated with overexpression of the mouse protein. - Rat Genome Database

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Gamma-synucleinopathy: neurodegeneration associated with overexpression of the mouse protein.

Authors: Ninkina, N  Peters, O  Millership, S  Salem, H  Van der Putten, H  Buchman, VL 
Citation: Ninkina N, etal., Hum Mol Genet. 2009 May 15;18(10):1779-94. Epub 2009 Feb 26.
RGD ID: 6478696
Pubmed: PMID:19246516   (View Abstract at PubMed)
PMCID: PMC2671987   (View Article at PubMed Central)
DOI: DOI:10.1093/hmg/ddp090   (Journal Full-text)

The role of alpha-synuclein in pathogenesis of familial and idiopathic forms of Parkinson's disease, and other human disorders known as alpha-synucleinopathies, is well established. In contrast, the involvement of two other members of the synuclein family, beta-synuclein and gamma-synuclein, in the development and progression of neurodegeneration is poorly studied. However, there is a growing body of evidence that alpha-synuclein and beta-synuclein have opposite neuropathophysiological effects. Unlike alpha-synuclein, overexpressed beta-synuclein does not cause pathological changes in the nervous system of transgenic mice and even ameliorates the pathology caused by overexpressed alpha-synuclein. To assess the consequences of excess expression of the third family member, gamma-synuclein, on the nervous system we generated transgenic mice expressing high levels of mouse gamma-synuclein under control of Thy-1 promoter. These animals develop severe age- and transgene dose-dependent neuropathology, motor deficits and die prematurely. Histopathological changes include aggregation of gamma-synuclein, accumulation of various inclusions in neuronal cell bodies and processes, and astrogliosis. These changes are seen throughout the nervous system but are most prominent in the spinal cord where they lead to loss of spinal motor neurons. Our data suggest that down-regulation of small heat shock protein HSPB1 and disintegration of neurofilament network play a role in motor neurons dysfunction and death. These findings demonstrate that gamma-synuclein can be involved in neuropathophysiological changes and the death of susceptible neurons suggesting the necessity of further investigations of the potential role of this synuclein in disease.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
Gliosis  ISOSncg (Mus musculus)6478696; 6478696 RGD 
Gliosis  IMP 6478696 RGD 
Nerve Degeneration  ISOSncg (Mus musculus)6478696; 6478696 RGD 
Nerve Degeneration  IMP 6478696 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Sncg  (synuclein, gamma)

Genes (Mus musculus)
Sncg  (synuclein, gamma)

Genes (Homo sapiens)
SNCG  (synuclein gamma)


Additional Information