Description
Product Usage Information
Western Blotting: 1:1000
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.
Protocol
Available protocols: Western Blotting
Specificity / Sensitivity
Senataxin (E9Z9F) Rabbit Monoclonal Antibody recognizes endogenous levels of total senataxin protein.
Species Reactivity: Human, Mouse, Rat, Monkey
Source / Purification
Monoclonal antibody is produced by immunizing animals with recombinant protein specific to the amino terminus of human senataxin protein.
Background
Senataxin is a ubiquitously expressed RNA-DNA helicase encoded by the gene. Senataxin is well known for resolving RNA-DNA hybrid structures known as R-loops, which can form during gene transcription when the template DNA strand hybridizes with nascent mRNA (1). Senataxin is also recruited to DNA double-strand breaks (DSBs) on active genes, where it promotes cell survival and prevents translocations (2). Although R-loops may positively regulate gene expression under certain conditions, unresolved R-loops can promote DNA damage and have been linked to neurodegenerative disorders (3). Indeed, senataxin mutations are the root cause of the neurological conditions ataxia with oculomotor apraxia type 2 (AOA2) and juvenile amyotrophic lateral sclerosis (ALS4) (4-7). Senataxin dysregulation has also been observed in several types of cancer, where it may play a tumor suppressive role (8). Interestingly, loss of senataxin has been found to cause accumulation of RNA-DNA hybrids in the cytoplasm, consequently promoting an innate immune response via cGAS and IRF3 (9). This innate immune activation could provide an additional link between senataxin dysfunction and disease (9).
Alternate Names
ALS4; Amyotrophic lateral sclerosis 4 protein; AOA2; bA479K20.2; DKFZp781B151; FLJ12840; FLJ43459; KIAA0625; Probable helicase senataxin; SCAN2; SCAR1; Sen1; SEN1 homolog; senataxin; SETX
