Anti-Senataxin / SETX

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. Senataxin antibody detects DNA/RNA helicase Senataxin, encoded by the SETX gene located on chromosome 9q34.13. Senataxin is a large nuclear helicase that unwinds DNA/RNA hybrids known as R-loops, thereby maintaining genomic stability during transcription and replication. It plays crucial roles in transcription termination, RNA processing, and DNA damage repair, linking transcriptional control with genome maintenance. Senataxin is expressed widely, with elevated levels in neurons, germ cells, and proliferative tissues where transcription-coupled DNA repair is highly active.Structurally, Senataxin is a 2677-amino-acid helicase belonging to the superfamily 1 (SF1) of DNA/RNA helicases. It contains a C-terminal helicase domain with conserved Walker A and B motifs for ATP binding and hydrolysis, an N-terminal region that mediates protein-protein interactions, and nuclear localization sequences. The helicase activity of Senataxin resolves R-loop structures formed during transcription, preventing collisions between transcription and replication machinery. Co-localization studies show Senataxin enriched in nuclear foci associated with sites of active transcription and DNA repair.Functionally, Senataxin maintains genome integrity by resolving R-loops and facilitating proper termination of RNA polymerase II transcription. It contributes to DNA double-strand break repair via homologous recombination and regulates transcription-coupled nucleotide excision repair. Senataxin also modulates RNA maturation by interacting with RNA processing factors such as XRN2 and the exosome complex. In neurons, it supports axonal maintenance and stress granule dynamics, while in germ cells, it regulates meiotic recombination and spermatogenesis.Mutations in the SETX gene are linked to several neurodegenerative and neuromuscular disorders, including ataxia with oculomotor apraxia type 2 (AOA2) and juvenile amyotrophic lateral sclerosis type 4 (ALS4). These conditions are characterized by progressive neurodegeneration and impaired DNA repair capacity. Senataxin deficiency leads to accumulation of R-loops, transcriptional stress, and genomic instability. Pathway associations include RNA processing, DNA damage response, and transcription regulation. During development, Senataxin expression supports neuronal differentiation and genome maintenance in post-mitotic cells.The Senataxin antibody from NSJ Bioreagents is a valuable reagent for studying transcription-coupled repair, RNA metabolism, and neurodegenerative disease mechanisms.