Humaan Adenylosuccinaat Lyase/ADSL antilichaam

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) .

Adenylosuccinate lyase, AMPS, ASASE, ASL

Human

Human

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

AT16C10

Unconjugated

ELISA, WB, ICC/IF, FACS

By protein-A affinity chromatography

1 mg/mL (determined by BCA assay)

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

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

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.

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

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.

Metabolism

NP_000017

P30566

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

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) .

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) .