. Author manuscript; available in PMC: 2012 Nov 1.

Published in final edited form as: Trends Neurosci. 2011 Sep 29;34(11):581–590. doi: 10.1016/j.tins.2011.08.003

Table 1.

Summary of selected transgenic mouse models of MSA

		PHENOTYPE
TRANSGENE	PROMOTER	NEUROPATHOLOGY	BEHAVIOR	REFS
Human α-syn	PLP	Dopaminergic cell loss in substantia nigra, loss of neurons in spinal cord and axonal degeneration. Delayed loss of cholinergic neurons and neurons in areas associated with autonomic failure. GCI-like inclusion comprised of insoluble α-syn, hyperphosphorylated at S129	Shorted stride length. The administration of 3NP in these mice results in progressive motor disability as assessed by the Bordeaux motor behavior scale, impaired rearing, hindlimb strength and pole test performance.	^52,55,58,105
Human α-syn	2',3'-cyclic nucleotide 3' phosphodiesterase (CNP)	Neuronal loss in hippocampus and cortex, demyelination and axonal degeneration. GCI-like inclusions of insoluble α-syn in addition to accumulation of α-syn in the neuropil	Impaired motor performance, as assessed in the rotarod and hanging wire tests	⁵⁴
Human α-syn	MBP	Loss of dopaminergic fibers in the basal ganglia, astrogliosis, loss of dendritic density, demyelination and mitochondrial alterations. Progressive accumulation of α-syn and S129 phosphorylated α-syn immunoreactive inclusions in oligodendrocytes along axonal tracts in brainstem, basal ganglia, cerebellum, corpus callosum and neocortex. The administration of 3NP in these mice enhances the oxidative modifications of α-syn	Impaired motor behavior as assessed by pole test and rotarod. Impaired olfaction.	^{53, 59, 84}