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

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG2a,k

AT1E11

Mouse

By protein-A affinity chromatography

Liquid. In phosphate-buffered saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol.

1mg/ml (lot specific)

Can be stored at 4 degree C.
For long term storage, aliquot and store at -20 degree C.
Avoid repeated freezing and thawing cycles.

Small volumes of anti-PRPS1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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).

Epigenomics

ELISA (EIA), Western Blot (WB), Immunocytochemistry (ICC), Immunofluorescence (IF)

Recommended starting dilution is 1:1000.

The cell lysate of 293T (30ug) were 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.

ICC/IF analysis of PRPS1 in HeLa cells line, stained with DAPI (Blue) for nucleus staining and monoclonal anti-human PRPS1 antibody (1:100) with goat anti-mouse IgG-Alexa fluor 488 conjugate (Green).

27,526 Da

phosphoribosyl pyrophosphate synthetase 1

ribose-phosphate pyrophosphokinase 1

Ribose-phosphate pyrophosphokinase 1

PRS-I??[Similar Products]

This gene encodes an enzyme that catalyzes the phosphoribosylation of ribose 5-phosphate to 5-phosphoribosyl-1-pyrophosphate, which is necessary for purine metabolism and nucleotide biosynthesis. Defects in this gene are a cause of phosphoribosylpyrophosphate synthetase superactivity, Charcot-Marie-Tooth disease X-linked recessive type 5 and Arts Syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Feb 2011]

Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.

Ishizuka T, et al. (1992) Biochim Biophsy Acta 24;1130 (2): 139-48; Kim HJ, et al. (2007) Arn J Hum Gernet 81 (3):552-8.

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.