Table 3.

Genetic modifiers of mutant ATXN3 toxicity.

Protein name	Function	Effect on toxicity	MJD model	Reference
ATXN3	DUB	suppressor	D. melanogaster	Warrick et al., 2005
		none	R. norvegicus	Alves et al., 2010
		none	M. musculus	Hubener et al., 2010
USP47	DUB	suppressor	D. melanogaster	Bilen et al., 2007
CHIP	Ub E3 ligase	suppressor	M. musculus	Williams et al., 2009
E4B	Ub E3 ligase	suppressor	HEK293T cells	Matsumoto et al., 2004
Ubiquitin	post-translational tag	suppressor	D. melanogaster	Bilen et al., 2007
VCP	AAA ATPase	suppressor	D. melanogaster	Boeddrich et al., 2006
HSP70	chaperone	suppressor	D. melanogaster	Warrick et al., 1999
			D. melanogaster	Bilen et al., 2007
HSP40	chaperone	suppressor	D. melanogaster	Bilen et al., 2007
αB-Crystallin	chaperone	suppressor	D. melanogaster	Bilen et al., 2007
HSPB8	chaperone	suppressor	HEK293T cells	Carra et al., 2010
			D. melanogaster
TPR2	chaperone	suppressor	D. melanogaster	Bilen et al., 2007
CRAG	Guanosine triphosphatase	suppressor	M. musculus	Torashima et al., 2008
Beclin-1	protein binding	suppressor	neuronal cells	Nascimento-Ferreira et al., 2011
			R. norvegicus
Bantam	microRNA	suppressor	D. melanogaster	Bilen et al., 2006
MBNL1	nucleic acid binding	suppressor	D. melanogaster	Li et al., 2008
IGF2BP2	mRNA binding	suppressor	D. melanogaster	Bilen et al., 2007
PABP	poly(A) RNA binding	suppressor	D. melanogaster	Lessing et al., 2008
UBXD2	UBX protein	suppressor	D. melanogaster	Bilen et al., 2007
UBXD8	UBX protein	suppressor	D. melanogaster	Bilen et al., 2007
Exportin1	protein transporter	suppressor	D. melanogaster	Bilen et al., 2007
HSF1	transcription factor	suppressor	C. elegans	Teixeira-Castro et al., 2011
DAF16	transcription factor	suppressor	C. elegans	Teixeira-Castro et al., 2011
NFAT5	transcription factor	suppressor	D. melanogaster	Bilen et al., 2007
ATF2	transcription factor	suppressor	D. melanogaster	Bilen et al., 2007
SIN3A	transcription regulator	suppressor	D. melanogaster	Bilen et al., 2007
TRIM71	transcription regulator	suppressor	D. melanogaster	Bilen et al., 2007
Dbr	zinc ion binding	suppressor	D. melanogaster	Bilen et al., 2007
ACOX1	palmitoyl-CoA oxidase	suppressor	D. melanogaster	Bilen et al., 2007
Sup35	yeast prion domain	suppressor	D. melanogaster	Li et al., 2007
UL97	HCMV kinase	supressor	HeLa cells	Tower et al., 2011
FBXL11	transcription regulator	enhancer	D. melanogaster	Bilen et al., 2007
ATXN2	RNA and protein binding	enhancer	D. melanogaster	Lessing et al., 2008