Researchers recently identified two proteins, HDBP1 and HDBP2, which bind to the promoter region of the Huntington gene. The promoter region of a gene is the place where RNA polymerase binds during transcription, the process by which DNA becomes RNA (which then goes through a process called translation to give rise to a protein). Proteins like HDBP1 and HDBP2 are called transcription factors because they bind to the promoter region, thereby promoting transcription. In this manner, the promoter region literally promotes the expression of a gene.
The highly conserved sequence at one end of the protein (a sequence of amino acid building blocks that has remained essentially unchanged throughout evolution) contributes to its ability to bind to the unique promoter region of the Huntington gene. HDBP1 and HDBP2 shuttle back and forth between the nucleus (central region) and cytoplasm of the cell and bind to the promoter region in order to promote the transcription of the Huntington gene.
Isolating these two proteins was a big step for researchers because, as transcription factors, they are key to regulating a critical part of the process that starts at the Huntington gene and ends in the huntingtin protein. With this new knowledge, scientists may someday be able to stop the shuttling activity of HDPB 1 and 2, thereby halting the transcription of the abnormal copy of the Huntington gene, the HD allele. By halting the transcription of the HD allele, this allele would no longer give rise to an altered form of the huntingtin protein, thus stopping the disease process of HD.
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