Ribose-phosphate pyrophosphokinase 1; PPRibP; Phosphoribosyl pyrophosphate synthase I; PRS-I; PRPS1

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

Polyclonal

Rabbit Ig

Rabbit

This PRPS1/PRPS2/PRPS3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 79-109 amino acids from the N-terminal region of human PRPS1/PRPS2/PRPS3.

Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Vial Concentration: 2 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw 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.

Phosphoribosylpyrophosphate synthetase (PRPS; EC 2.7.6.1) catalyzes the phosphoribosylation of ribose 5-phosphate to 5-phosphoribosyl-1-pyrophosphate, which is necessary for the de novo and salvage pathways of purine, pyrimidine, and pyridine biosynthesis. By PCR of human lymphoblast mRNA using primers based on the cDNA sequence of rat PRS I (Prps1), Roessler et al. (1990) isolated a partial human PRPS1 cDNA. They used this partial cDNA to screen lymphoblast cDNA libraries and isolated additional cDNAs corresponding to the entire PRPS1 coding region. The deduced PRPS1 protein has 318 amino acids.

Cancer; Cell Biology; Metabolism

Western Blot (WB), ELISA (EIA)

WB~~1:1000

The anti-PRPS1/2/3 Pab is used in Western blot to detect PRPS1/2/3 in mouse kidney tissue lysate (Lane 1) and Hela cell lysate (Lane 2).

Role of PPP during MSC transformation. (A) G6PD activity decreased during MSC transformation (?, P=0.0132; t test). Western blot confirms the down-regulation of G6PD and the up-regulation of PRPS at the late stages of transformation.

34834

phosphoribosyl pyrophosphate synthetase 1

ribose-phosphate pyrophosphokinase 1

Ribose-phosphate pyrophosphokinase 1

PRS-I??[Similar Products]

PRPS1_HUMAN

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]

PRPS1: Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis. Defects in PRPS1 are the cause of phosphoribosylpyrophosphate synthetase superactivity (PRPS1 superactivity); also known as PRPS-related gout. It is a familial disorder characterized by excessive purine production, gout and uric acid urolithiasis. Defects in PRPS1 are the cause of Charcot-Marie-Tooth disease X-linked recessive type 5 (CMTX5); also known as optic atrophy-polyneuropathy-deafness or Rosenberg-Chutorian syndrome. CMTX5 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy. Defects in PRPS1 are the cause of ARTS syndrome (ARTS); also known as fatal ataxia X-linked with deafness and loss of vision. ARTS is a disorder characterized by mental retardation, early-onset hypotonia, ataxia, delayed motor development, hearing impairment, and optic atrophy. Susceptibility to infections, especially of the upper respiratory tract, can result in early death. Defects in PRPS1 are the cause of deafness X-linked type 1 (DFNX1); also known as congenital sensorineural deafness X-linked 2 (DFN2). It is a form of deafness characterized by progressive, severe-to-profound sensorineural hearing loss in males. Females manifest mild to moderate hearing loss. Belongs to the ribose-phosphate pyrophosphokinase family.

Protein type: Kinase, other; Nucleotide Metabolism - purine; EC 2.7.6.1; Carbohydrate Metabolism - pentose phosphate pathway

Chromosomal Location of Human Ortholog: Xq22.3

Cellular Component: cytosol

Molecular Function: ribose phosphate diphosphokinase activity; protein homodimerization activity; magnesium ion binding; kinase activity; ATP binding

Biological Process: nervous system development; carbohydrate metabolic process; hypoxanthine biosynthetic process; ribonucleoside monophosphate biosynthetic process; 5-phosphoribose 1-diphosphate biosynthetic process; pathogenesis; purine nucleotide biosynthetic process; phosphorylation; purine base metabolic process; pyrimidine nucleotide biosynthetic process

Disease: Charcot-marie-tooth Disease, X-linked Recessive, 5; Arts Syndrome; Phosphoribosylpyrophosphate Synthetase Superactivity

Roessler, B. J., et al. Nucleic Acids Res. 18: 193 (1990).

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