Exalpha Biologicals, Inc.

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FIX&PERM Cell Fixation and Permeabilization Kit

Flow cytometric analyses with monoclonal antibodies were so far mainly restricted to cell surface molecules. Intracellular structures such as cytoplasmic or nuclear enzymes, oncoproteins, cytokines, immunoglobulins etc. were largely excluded from such studies. Also excluded from flow cytometric studies were cytoplasmic localizations of well-established membrane molecules like CD3 and CD22, which, in their cytoplasmic form, are the most reliable lineage markers in undifferentiated leukemia. With the FIX&PERM® Kit flow cytometric analysis of intracellular antigens has become as easy as surface antigen studies. The only prerequisite is the availability of suitable antibody conjugates. Most of the available monoclonal antibody conjugates can be used with the FIX&PERM® Kit, some determinants are sensitive, however, to the fixation step involved. This and the optimal fixation time have to be tested for each reagent.

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Exalpha Biologicals, Inc.

Aprataxin

  • Product Code: X2725P
  • Size: 100 µg
  • Availability: In Stock In Stock
  • Price (USD): $355

Cat #

X2725P		 Quantity:      

Data Sheet

Product Name

Aprataxin

Synonyms

Forkhead-associated domain histidine triad-like protein; FHA-HIT; APTX; AXA1

Host/Source

Rabbit

Product Type

Antigen Immunoaffinity Purified Polyclonal

Reactivity

Human, Rat, Mouse

Applications

Western blotting, ELISA

Purification

Antigen Immunoaffiinity Purification

Size

100 µg

Price (USD)

$355

Background

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.

Immunogen

Synthetic peptide derived from the human aprataxin protein.

Positive Control

Human lung tissue

Formulation

Provided as solution in phosphate buffered saline with 0.08% sodium azide

Customer Storage

Product should be stored at -20°C. Aliquot to avoid freeze/thaw cycles

Target Molecular Weight

41 kDa

Product Image

Image Legend

Immunohistochemical staining of normal human lung tissue using Aprataxin antibody (Cat. No. X2725P) at 10 µg/ml.

Database Links:

SwissProtQ7TQC5Mouse
SwissProtQ8K4H4Rat
SwissProtQ7Z2E3Human

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.