Humaan PRPS1-antilichaam

PRPS1 is an enzyme that catalyzes the phosphoribosylation of ribose 5-phosphate to 5-phosphoribosyl-1-pyrophosphate, which is necessary for purine metabolism and nucleotide biosynthesis. A mutation in PRPS1 may result in PRPS superactivity, a disease characterized by gout and the overproduction of purine nucleotides, uric acid and PRPP. PRPS1 mutations can also lead to a reduction in PRPS1 activity resulting in ARTS syndrome or CMTX5 (Charcot-Marie-Tooth disease X-linked recessive type 5) .

Ribose-phosphate pyrophosphokinase 1, ARTS, CMTX5, PPRibP, PRSI, DFN2, DFNX1, Ribose-phosphate pyrophosphokinase 1 EC 2.7.6.1, KIAA0967, Phosphoribosyl pyrophosphate synthetase I, PRS I, PRS-I, Ribose phosphate pyrophosphokinase I

Human

Human

Recombinant human PRPS1 (1-318aa) purified from E. coli

AT1E11

Unconjugated

ELISA, WB, ICC/IF

By protein-A affinity chromatography

1 mg/mL (determined by BCA assay)

PRPS1, ATGA0279-10 µg, ATGA0279-20 µg, ATGA0279-50 µg, ATGA0279-100 µg, ATGA0279-250 µg, ATGA0279-500 µg, ATGA0279-1 mg, ATGA0279-10, ATGA0279-20, ATGA0279-50, ATGA0279-100, ATGA0279-250, ATGA0279-500, ATGA0279-1

Ishizuka T, et al. (1992) Biochim Biophsy Acta 241130 (2) : 139-48.; ; Kim HJ, et al. (2007) Arn J Hum Gernet 81 (3) :552-8.

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 and ICC/IF analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results.

Epigenomics (Transcription & Translation)

NP_002755

P60891

The cell lysate (40ug) was resolved by SDS-PAGE, transferred to PVDF membrane and probed with anti-human PRPS1 antibody (1:1000) . Proteins were visualized using a goat anti-mouse secondary antibody conjugated to HRP and an ECL detection system.; ; Lane 1.: 293T cell lysate

ICC/IF analysis of PRPS1 in HeLa cells. The cell was stained with ATGA0279 (1:100) . The secondary antibody (green) was used Alexa Fluor 488. DAPI was stained the cell nucleus (blue) .