. Author manuscript; available in PMC: 2013 May 16.

Published in final edited form as: Semin Neurol. 2012 Jan 21;31(5):470–483. doi: 10.1055/s-0031-1299786

Table 2.

Polyglutamine proteins in epigenetic and transcriptional regulation

Disease	polyQ-proteins	Functions related to transcription/epigenetics	Other cellular functions
DRPLA	Atrophin-1	Transcriptional repressor¹⁴⁸
Huntington Disease	Huntingtin	TF interacting protein¹²⁰ DNA binding protein¹²² CBP interacting protein^112,114,129	Vesicle Transport Signal Transduction
SBMA	Androgen receptor	Nuclear receptor¹⁴⁹ and transcription factor
SCA1	Ataxin-1	Transcriptional regulation, histone acetylation^95–97
SCA2	Ataxin-2	Transcriptional co-activator¹⁵⁰	RNA binding¹⁵¹
SCA3	Ataxin-3	Transcriptional repressor¹⁵²	Deubiquitinase¹⁵³
SCA6	CACNA1A	C-terminal PolyQ fragment traffics to nucleus¹⁵⁴	P/Q type Calcium channel¹⁵⁵
SCA7	Ataxin-7	Coactivator for STAGA histone acetylation complex^102,104
SCA17	TBP	General transcription factor¹⁵⁶

Abbreviations: DRPLA- Dentatorubral pallidoluysian atrophy; TF- transcription factor; CBP- CREB binding protein; SBMA- Spinal Bulbar Muscular Atrophy (a.k.a. Kennedy’s Disease); SCA-Spinocerebellar Ataxiaf; PolyQ- polyglutamine; STAGA- SPT3-TAFII31-GCN5L acetylase complex; TBP- TATA binding protein