Rabbit anti-Human, Mouse PRPS1, PRPS2, PRPS3 Polyclonal Antibody | anti-PRPS1 antibody

PRPS1, PRPS2, PRPS3, NT (Ribose-phosphate pyrophosphokinase 1, PPRibP, Phosphoribosyl pyrophosphate synthase I) (Biotin)

Cat. Num. AAA6337668

• Gene Names: PRPS1; ARTS; DFN2; PRSI; CMTX5; DFNX1; PRS-I; PPRibP
• Reactivity: Human, Mouse
• Applications: Western Blot, ELISA
• Purity: Purified by Protein G Affinity Chromatography.
• Synonyms: PRPS1; PRPS2; PRPS3; Polyclonal Antibody; NT (Ribose-phosphate pyrophosphokinase 1; PPRibP; Phosphoribosyl pyrophosphate synthase I) (Biotin); Ribose-phosphate pyrophosphokinase 1; Phosphoribosyl pyrophosphate synthase I; anti-PRPS1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein G Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-PRPS1 antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: PRPS1/PRPS2/PRPS3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 87~116 amino acids from the N-terminal region of human PRPS1/2/3.
• Conjugate: Biotin
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(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).)

Western Blot (WB)

(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.)

Related Product Information for anti-PRPS1 antibody

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.

Product Categories/Family for anti-PRPS1 antibody

Antibodies; Enzymes; Synthase

References

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

NCBI and Uniprot Product Information

• NCBI GI #: 324711015
• NCBI GeneID: 5631
• NCBI Accession #: NP_001191331.1
• NCBI GenBank Nucleotide #: NM_001204402.1
• UniProt Accession #: P60891; P09329; B1ALA8; B2R6T7; B4DNL6; D3DUX6
• Molecular Weight: 27,526 Da
• NCBI Official Full Name: ribose-phosphate pyrophosphokinase 1 isoform 2
• NCBI Official Synonym Full Names: phosphoribosyl pyrophosphate synthetase 1
• NCBI Official Symbol: PRPS1
• NCBI Official Synonym Symbols: ARTS; DFN2; PRSI; CMTX5; DFNX1; PRS-I; PPRibP
• NCBI Protein Information: ribose-phosphate pyrophosphokinase 1; dJ1070B1.2 (phosphoribosyl pyrophosphate synthetase 1); deafness 2, perceptive, congenital; deafness, X-linked 2, perceptive, congenital; phosphoribosyl pyrophosphate synthase I; ribose-phosphate diphosphokinase 1
• UniProt Protein Name: Ribose-phosphate pyrophosphokinase 1
• UniProt Gene Name: PRPS1
• UniProt Synonym Gene Names: PRS-I
• UniProt Entry Name: PRPS1_HUMAN

NCBI Description

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]

Uniprot Description

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

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; hypoxanthine biosynthetic process; carbohydrate metabolic process; 5-phosphoribose 1-diphosphate biosynthetic process; ribonucleoside monophosphate 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