Ataxin-1 (ATXN1) is involved in the formation and regulation of multimeric protein complexes within the nucleus []. It is the causative factor for spinocerebellar ataxia type 1 (SCA1), which manifests in poor co-ordination of hand and feet movement []. ATXN1 is a transcription repressor in the Notch signalling pathway []and colocalizes with the deubiquitinating enzyme USP7 in the nucleus [].
UVSSA is part of a UV-induced ubiquitinated protein complex involved in transcription-coupled nucleotide excision repair (TC-NER) in response to UV damage. It stabilise the TC-NER master organizing protein ERCC6 (also known as CSB) by delivering the deubiquitinating enzyme USP7 to TC-NER complexes [].
This entry includes Ataxin1 and Ataxin1-like proteins. They are components of the Notch signalling pathway involved in transcriptional repression [].Ataxin-1 (ATXN1) is involved in the formation and regulation of multimeric protein complexes within the nucleus []. It is the causative factor for spinocerebellar ataxia type 1 (SCA1), which manifests in poor co-ordination of hand and feet movement []. ATXN1 is a transcription repressor in the Notch signalling pathway []and colocalizes with the deubiquitinating enzyme USP7 in the nucleus [].
In mouse embryonic stem cells (mESCs), the transcriptional network can be divided into three functionally distinct modules: Polycomb, Core, and Myc, and the Polycomb module represses developmental genes []. This family includes the polycomb repressive complex 2-associated factor EPOP (Elongin BC and Polycomb Repressive Complex 2 (PRC2) Associated Protein, also termed C17orf96, esPRC2p48, E130012A19Rik), a scaffold protein expressed in the inner cell mass of the mouse blastocyst serving as a bridging partner between the PRC2/EED-EZH2 complex and the elongin BC complex, and fine-tuning the transcriptional status of Polycomb group (PcG) target genes in embryonic stem cells. Both EPOP and Elongin BC are required to maintain low levels of expression at PRC2 genomic targets []. EPOP interacts with the H2B deubiquitinase USP7 to modulate transcriptional processes in mESCs similar to MYC [].Another member of the family is the uncharacterized SKI/DACH domain-containing protein 1.
