The region of interest was narrowed down to a 17 bp sequence located between 4,944 bp and 4,961 bp upstream of exon 1 of the FXN gene. The removal of the 17 bp sequence resulted in a reduction of gene expression of 45% and 67% in HeLa and BE -M17 cell lines, respectively. The 17 bp sequence is located within conserved non-coding region 1 and it is sufficient and necessary for maximal FXN gene expression. This finding in the luciferase system was further supported by the genomic reporter assay system. Deletion of the 17 bp region in the context of the entire human FXN gene in the BAC reporter clearly demonstrated a reduction in EGFP expression, by flow cytometry analysis and fluorescent microscopy, when LY2157299 TGF-beta inhibitor compared to the control dual reporter unmodified plasmid. Furthermore, the EGFP expression of this construct was comparable to that observed where the entire conserved region 1 was deleted. It was therefore evident that the absence of this region in the BAC genomic reporter assay resulted in a decrease in FXN gene expression and the presence of this region in the small Niraparib plasmid reporter system resulted in an increase in gene expression. In an attempt to comprehensively identify regulatory element located within the 17 bp sequence, in silico approaches were undertaken. A binding site for the Oct-1 transcription factor was the most likely candidate identified by three different search tools. Oct-1 is known to bind to an 8-bp sequence termed an octamer motif. Mice lacking Oct-1 die during early development indicating that the presence of this transcription factor is essential for life. Unlike other members of the POU protein family, this transcription factor is expressed ubiquitously and no specific temporal or spatial pattern is observed. Oct-1 is expressed in all eukaryotic cells and regulates, either positively or negatively, the expression of a variety of genes. This ability is attributed to its flexibility in binding DNA as a monomer, homodimer, or heterodimer. This regulatory factor has also been shown to be important for tissue and cell-specific transcription as well as the transcription of a number of housekeeping genes. Oct-1 has also been shown to participate in recruiting preinitiation complexes in the promoter region of genes lacking a TATA box by functionally substituting the role of transcription binding protein. In the case of the FXN gene, this finding is very relevant as there is no TATA box sequence located within the vicinity of the FXN promoter region. The candidate Oct-1 sequence in the 17 bp region did not exactly match the human Oct-1 binding site consensus sequence GCAT). The missing nucleotide has been found not to be critical for Oct-1 binding. It has been found that the essential nucleotides for Oct-1 binding are located at the 59 end of the sequence.