Transgene: human wild-type α-synuclein (α-syn) cDNA
Promoter: neuron specific Thy-1
Mouse Strain: C57BL/6
Neuropathological Analysis:
Expression of transgenic (Tg) α-syn largely paralleled that of endogenous mouse
α-syn, with the exception of the first postnatal week, when small but significant
amounts of endogenous, but no Tg α-syn, was seen (as expected for Thy1 cassette).
Tg expression was up to three times that of endogenous α-syn expression.
The wt human α-syn was found in neuronal cell bodies and was also anterogradely
transported to synapses, in contrast to the endogenous mouse α-syn, which was
not observed in the somal compartment. Abnormal Tg α-syn-positive neurites,
a characteristic of Lewy body disease, were seen, occasionally emanating from neuronal
cell body. Tg α-syn, but not endogenous α-syn (or nonamyloidogenic β-syn),
was found in detergent-insoluble fractions of fresh tissue.
Patents:
Primary:
Kahle PJ, Neumann M, Ozmen L, Muller V, Odoy S, Okamoto M, Jacobsen H, Iwatsubo
T, Trojanowski JQ, Takaashi H, Wakabayashi K, Bogdanovic N, Riederer P, Kretzschmar
HA, Haass C. Selective insolubility of "-synuclein in human Lewy body diseases is
recapitulated in a transgenic mouse model. Am J Pathol. 2001 Dec;159(6):2215-25.
Abstract
.
Secondary:
Kahle PJ, Neumann M, Ozmen L, Muller V, Jacobsen H, Schindzielorz A, Masayasu O,
Leimer U, van der Putten H, Probst A, Kremmer E, Kretzschmar HA, Haass C. Subcellular
localization for wild-type and Parkinson's disease-associated mutant "-synuclein
in human transgenic mouse brain. J Neurosci. 2000 Sep 1;20(17):6365-73.
Abstract
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