Table 1.

SUMOylation and neurodegenerative disorders.

		Implicated SUMO substrates	References
Neuronal intranuclear inclusion disorder		HDAC4	McFadden et al. (2005), Pountney et al. (2003), Takahashi-Fujigasaki et al. (2006)
		PML
		RanGAP1
		NSF*
		Ubc18-1*
		Dynamin-1*
		HSP90*
Polyglutamine diseases	Huntington’s	Huntingtin	Steffan et al. (2004)
	SBMA	Androgen receptor	Poukka et al. (2000)
	DRPLA	Unknown	Terashima et al. (2002)
	SCA Type I	Ataxin-1	Riley et al. (2005), Ryu et al. (2010)
	SCA Type VII	Ataxin-7	Janer et al. (2010)
Alzheimer’s disease		APP	Gocke et al. (2005), Zhang and Sarge (2008)
		Tau	Dorval and Fraser (2006)
α-Synucleinopathies	Parkinson’s	α-Synuclein	Dorval and Fraser (2006)
		DJ-1	Shinbo et al. 2006
	MSA	α-Synuclein	Dorval and Fraser (2006)
	DLB	α-Synuclein	Dorval and Fraser (2006)
Hypoxia, ischemia and cellular stress		Various unknown	Cimarosti et al. (2008), Yang et al. (2008a,b)

SUMOylation has been associated with multiple neurological disorders and a number of disease-associated proteins have been reported to be SUMO substrates. Proteins marked with an asterisk (*) have been isolated from SUMO-positive inclusions but have not been directly shown to be SUMOylated.

Abbreviations: SBMA, spinal and bulbar muscular atrophy; DRPLA, dentatorubral-pallidoluysian atrophy; SCA, spinocerebellar ataxia; APP, amyloid precursor protein; MSA, multiple system atrophy; DLB, demetia with Lewy bodies.