Product Name :
Rabbit anti Human Aprataxin
Description :
| Product Type Polyclonal Antibody | Units 100 µg | Host Rabbit | Species Reactivity Human Mouse Rat | Application ELISA Western Blotting
Background :
Immunogen: Synthetic peptide derived from the human aprataxin protein.
Source :
DNA-binding protein involved in single-strand DNA break repair, double-strand DNA break repair and base excision repair. Resolves abortive DNA ligation intermediates formed either at base excision sites, or when DNA ligases attempt to repair non-ligatable breaks induced by reactive oxygen species. Catalyzes the release of adenylate groups covalently linked to 5′-phosphate termini, resulting in the production of 5′-phosphate termini that can be efficiently rejoined. Also able to hydrolyze adenosine 5′-monophosphoramidate (AMP-NH2) and diadenosine tetraphosphate (AppppA), but with lower catalytic activity. Protein is widely expressed. Defects in APTX are the cause of ataxia-oculomotor apraxia syndrome, an autosomal recessive syndrome characterized by early-onset cerebellar ataxia, oculomotor apraxia, early areflexia and late peripheral neuropathy. Also a cause of coenzyme Q10 deficiency. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy. Coenzyme Q10 deficiency due to APTX mutations is typically associated with cerebellar ataxia. Synonyms: Forkhead-associated domain histidine triad-like protein; FHA-HIT; APTX; AXA1 <
Product :
Product Form: Unconjugated Formulation: Provided as solution in phosphate buffered saline with 0.08% sodium azide Purification Method: Ammonium Sulfate Precipitation Concentration: See vial for concentration
Specificity :
Applications :
Antibody can be used for Western blotting (1-5 mg/ml). Optimal concentration should be evaluated by serial dilutions. Functional Analysis: Western Blotting Positive Control: Human lung tissue
Storage :
Product should be stored at -20°C. Aliquot to avoid freeze/thaw cycles Product Stability: See expiration date on vial Shipping Conditions: Ship at ambient temperature, freeze upon arrival
Caution :
This product is intended FOR RESEARCH USE ONLY, and FOR TESTS IN VITRO, not for use in diagnostic or therapeutic procedures involving humans or animals. It may contain hazardous ingredients. Please refer to the Safety Data Sheets (SDS) for additional information and proper handling procedures. Dispose product remainders according to local regulations.This datasheet is as accurate as reasonably achievable, but our company accepts no liability for any inaccuracies or omissions in this information.
References :
1. Ahel, I., et al. ‘The neurodegenerative disease protein aprataxin resolves abortive DNA ligation intermediates.’ Nature 2006, 443, 713-716. 2. Kijas, A.W., et al. ‘Aprataxin forms a discrete branch in the HIT (histidine triad) superfamily of proteins with both DNA/RNA binding and nucleotide hydrolase activities.’ J. Biol. Chem. 2006, 281, 13939-13948. 3. Rass, U., et al. ‘Actions of aprataxin in multiple DNA repair pathways.’ J. Biol. Chem. 2007, 282, 9469-9474. 4. Rass, U., et al. ‘Molecular mechanism of DNA deadenylation by the neurological disease protein aprataxin.’ J. Biol. Chem. 2008, 283, 33994-34001.
Related websites: https://www.medchemexpress.com/antibodies.html
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