Product nameAnti-Aprataxin antibody
See all Aprataxin primary antibodies
DescriptionRabbit polyclonal to Aprataxin
Tested applicationsSuitable for: WB, IHC-P, ICC/IFmore details
Species reactivityReacts with: Human
Predicted to work with: Cow
Recombinant fragment, corresponding to a region within amino acids 1-356 of Human Aprataxin (Uniprot ID: Q7Z2E3)
- 293T, A431, H1299, HepG2, MOLT4 and Raji cell lysates; HeLa cells; Human A549 xenograft tissue.
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 1.21% Tris, 0.75% Glycine, 20% Glycerol
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab154413 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/3000. Predicted molecular weight: 41 kDa.|
|IHC-P||1/100 - 1/1000. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. Antigen retrieval also possible using Tris-EDTA buffer (pH8.0).|
|ICC/IF||1/100 - 1/1000.|
FunctionDNA-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-NH(2)) and diadenosine tetraphosphate (AppppA), but with lower catalytic activity.
Tissue specificityWidely expressed. In brain, it is expressed in the posterior cortex, cerebellum, hippocampus and olfactory bulb. Isoform 1 is highly expressed in the cerebral cortex and cerebellum, compared to isoform 2.
Involvement in diseaseDefects in APTX are the cause of ataxia-oculomotor apraxia syndrome (AOA) [MIM:208920]. AOA is an autosomal recessive syndrome characterized by early-onset cerebellar ataxia, oculomotor apraxia, early areflexia and late peripheral neuropathy.
Defects in APTX are a cause of coenzyme Q10 deficiency (COQ10D) [MIM:607426]. 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.
Sequence similaritiesContains 1 C2H2-type zinc finger.
Contains 1 FHA-like domain.
Contains 1 HIT domain.
DomainThe histidine triad, also called HIT motif, forms part of the binding loop for the alpha-phosphate of purine mononucleotide.
The FHA-like domain mediates interaction with NCL; XRCC1 and XRCC4.
The HIT domain is required for enzymatic activity.
The C2H2-type zinc finger mediates DNA-binding.
Cellular localizationNucleus > nucleoplasm. Nucleus > nucleolus. Upon genotoxic stress, colocalizes with XRCC1 at sites of DNA damage. Colocalizes with MDC1 at sites of DNA double-strand breaks. Interaction with NCL is required for nucleolar localization.
- Information by UniProt
- AOA 1 antibody
- AOA antibody
- AOA1 antibody
All lanes : Anti-Aprataxin antibody (ab154413) at 1/1000 dilution
Lane 1 : HeLa whole cell lysate
Lane 2 : HepG2 whole cell lysate
Lysates/proteins at 30 µg per lane.
Predicted band size: 41 kDa
10% SDS PAGE
Immunofluorescence analysis of paraformaldehyde-fixed HeLa cells, labeling Aprataxin using ab154413 at a 1/500 dilution.
Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human A549 xenograft tissue, labeling Aprataxin using ab154413 at a 1/100 dilution.
ab154413 has not yet been referenced specifically in any publications.