Human Adenylosuccinate Lyase/ADSL antibody

Adenylosuccinate lyase, also known as ADSL, is an enzyme that converts adenylosuccinate to AMP and fumarate as part of the purine nucleotide cycle. Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency) . ADSL deficiency is an autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado) .

Product Specifications

Product Name Alternative

Adenylosuccinate lyase, AMPS, ASASE, ASL

Host

Mouse

Antigen Species

Human

Reactivity

Human

Immunogen

Recombinant human ADSL (1-484aa) purified from E. coli

Clonality

Monoclonal

Isotype

IgG1 κ

Clone

AT16C10

Conjugation

Unconjugated

Applications

ELISA, WB, ICC/IF, FACS

Purification Method

By protein-A affinity chromatography

Concentration

1 mg/mL (determined by BCA assay)

Additionnal Information

ADSL, ATGA0400-10 µg, ATGA0400-20 µg, ATGA0400-50 µg, ATGA0400-100 µg, ATGA0400-250 µg, ATGA0400-500 µg, ATGA0400-1 mg, ATGA0400-10, ATGA0400-20, ATGA0400-50, ATGA0400-100, ATGA0400-250, ATGA0400-500, ATGA0400-1

References & Citations

Marie S., et al. (2002) Am J Hum Genet. 71: 14-21. ; ; Kmoch S., et al. (2000) Hum Mal Genet. 9: 1501-1513.;

Storage Conditions

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Formulation

Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol

Applications Notes

The antibody has been tested by ELISA, Western blot, ICC/IF and FACS analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results.

Scientific Category

Metabolism

NCBI Accession Number

NP_000017

Uniprot Accession Number

P30566

WB Description

The Recombinant Human ADSL (20ng) and Cell lysates (40ug) were resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human ADSL antibody (1:1000) . Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system. ; ; Lane 1. : Recombinant Protein ; ; Lane 2. : HeLa cell lysate ; ; Lane 3. : 293T cell lysate ; ; Lane 4. : Jurkat cell lysate ; ; Lane 5. : HepG2 cell lysate ; ; Lane 6. : A549 cell lysate ; ; Lane 7. : MCF7 cell lysate

FACS Description

Flow cytometry analysis of ADSL in Hep3B cell line, staining at 2-5ug for 1x10^6cells (red line) . The secondary antibody used goat anti-mouse IgG Alexa fluor 488 conjugate. Isotype control antibody was mouse IgG (black line) .

IF Description

ICC/IF analysis of ADSL in Hep3B cells. The cell was stained with ATGA0400 (1:100) . The secondary antibody (green) was used Alexa Fluor 488. DAPI was stained the cell nucleus (blue) .