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Ataxin‐1 and Brother of ataxin‐1 are components of the Notch signalling pathway
Author(s) -
Tong Xin,
Gui Hongxing,
Jin Feng,
Heck Bryan W,
Lin Peihui,
Ma Jianjie,
Fondell Joseph D,
Tsai ChihCheng
Publication year - 2011
Publication title -
embo reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.584
H-Index - 184
eISSN - 1469-3178
pISSN - 1469-221X
DOI - 10.1038/embor.2011.49
Subject(s) - notch signaling pathway , transcription factor , microbiology and biotechnology , biology , notch proteins , genetics , signal transduction , gene
Ataxin‐1 (ATXN1), a causative factor for spinocerebellar ataxia type 1 (SCA1), and the related Brother of ATXN1 (BOAT1) are human proteins involved in transcriptional repression. So far, little is known about which transcriptional pathways mediate the effects of ATXN1 and BOAT1. From our analyses of the properties of BOAT1 in Drosophila and of both proteins in mammalian cells, we report here that BOAT1 and ATXN1 are components of the Notch signalling pathway. In Drosophila , BOAT1 compromises the activities of Notch. In mammalian cells, both ATXN1 and BOAT1 bind to the promoter region of Hey1 and inhibit the transcriptional output of Notch through direct interactions with CBF1, a transcription factor that is crucial for the Notch pathway. Our results suggest that, in addition to their involvement in SCA1, ATXN1 and BOAT1 might participate in several Notch‐controlled developmental and pathological processes.