Name/
Symbol |
Strain Name |
Transgene/
Promoter and Regulatory Elements |
Genetic Background |
Behavioral
Phenotype |
Neurological
Characteristics |
Patents/
Availability |
Primary Citation |
A53T αsynuclein PRP transgenic-/-
(See JAX datasheet)Symbol: SNCA Posted 5/19/04 |
B6;C3H-Tg(SNCA)83Vle/J |
Mouse Prnp, prion protein promoter/ human α-synuclein A53T mutation cDNA /B6C3H mouse eggs. |
Origin: Backcross N?+N1 |
Homozygous mice are viable, fertile and normal in size. By 16 months display severe motor phenotype. Lax grooming, weight loss and diminished mobility precede movement impairment, partial limb paralysis, trembling and inability to stand. |
8 to 12 months widely distributed α-synuclein inclusions, with dense accumulation in the spinal cord, brainstem, cerebellum and thalamus. This parallels the onset of the motor impairment phenotype. Axons and myelin sheaths exhibit progressive ultrastructural degeneration. |
Available
The Jackson Lab: Stock 004479 |
Giasson et al., 2002 |
| Alpha Synuclein |
|
Human alpha-synuclein cDNA with A53T mutation/
Thy1 gene promoter |
C57BL/6 |
An early-onset (>3 weeks of age) and a progressive decline of motor function caused by severe degeneration of neuromuscular junctions. |
Increased levels of a-synuclein protein in brain found in neurons throughout the telencephalon, brainstem, and spinal cord. Also showed astrocytic gliosis and microglial activation. Many of the cells showed diffuse perikaryal a-synuclein staining. Intense staining with the Campbell-Switzer pyridine silver, a routine and sensitive method used to detect Lewy-type changes in human brain tissue. |
Unpatented |
van der Putten H et al., 2000;
Sommer B et al., 2000 |
| (PLP)-h[wt] αSYN Symbol: αSYN Updated 4/14/05 |
|
Human wt αSYN / proteolipid protein promoter (PLP)/injected into C57BL/6 DBA/2 fertilized eggs. |
Origin: C57BL/6. Backcross generation N7 |
|
Transgenic SYN was detected in brain white matter and oligodendrocytes, detergent insoluble. Hyperphosphorylation at S129 of SYN in the tg mice. |
Contact Philipp Kahle
at pkahle@pbm.med.uni-muenchen.de
|
Kahle et al., 2002 |
| (TH)-h[A30P] SYN Symbol: αSYN Posted 7/9/04 |
|
human mutant [A30P] SYN sequence was cloned into a cassette with 4.5-kb 5' flanking DNA from rat TH gene promotor/Injected DBA X C57BL/6 eggs. |
Origin: C57BL/6; Backcrossed to 4 generations |
No motor disability |
Tg mice showed somal and neuritic accumulation of transgenic A30P SYN in TH-positive neurones in the substantia nigra. No difference in the number of TH-positive neurones or concentrations of catecholamines between tg mice and non-tg littermates. |
Contact Philipp Kahle
at pkahle@pbm.med.uni-muenchen.de
|
Rathke-Hartlieb et al., 2001 |
| (Thy1)-h[A30P] SYN Updated 4/14/05 |
|
The amplified human [wt]Alpha-SYN-coding sequence with A30P mutation/ neuron specific Thy1 promoter |
Backcrossed N > 8 generations C57BL/6J, (2002) |
No overt motor abnormalities to 1 year of age. Shows age-gene-dose dependent lethal locomotor deterioration along with Lewy pathology. |
Mutant hα-SYN found in synapses, and positive neurites exhibit Lewy body
characteristics. Shows age- and gene dose- dependent development of Lewy pathology.
|
Unpatented Contact: Philipp Kahle |
Kahle et al., 2000 |
| (Thy1)-h[wt] αSYN Updated 11/4/03 |
|
human wild-type α-SYN cDNA |
C57BL/6 |
None listed. |
hα-SYN initially undetectable but then increased to plateau at 1 month, expression was 3 fold of endogenous α-SYN. Abnormal Tg α-syn-positive neurites were seen. Tg α-syn found in detergent-insoluble tissue fractions. |
Contact: Philipp Kahle |
Kahle et al., 2001 |
Home
