Ataxin‐1 and Brother of ataxin‐1 are components of the Notch signalling pathway
Copyright policy )Ataxin‐1 and Brother of ataxin‐1 are components of the Notch signalling pathway
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.
- Rutgers, The State University of New Jersey United States
Chromatin Immunoprecipitation, Nuclear Proteins, Cell Cycle Proteins, Nerve Tissue Proteins, Polymerase Chain Reaction, Cell Line, Mice, Ataxins, Microscopy, Fluorescence, Immunoglobulin J Recombination Signal Sequence-Binding Protein, Basic Helix-Loop-Helix Transcription Factors, Animals, Drosophila Proteins, Humans, Immunoprecipitation, Drosophila, RNA, Small Interfering, Ataxin-1, DNA Primers, Plasmids
Chromatin Immunoprecipitation, Nuclear Proteins, Cell Cycle Proteins, Nerve Tissue Proteins, Polymerase Chain Reaction, Cell Line, Mice, Ataxins, Microscopy, Fluorescence, Immunoglobulin J Recombination Signal Sequence-Binding Protein, Basic Helix-Loop-Helix Transcription Factors, Animals, Drosophila Proteins, Humans, Immunoprecipitation, Drosophila, RNA, Small Interfering, Ataxin-1, DNA Primers, Plasmids
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