Anti-PRPS1/2/1L1 antibody

Anti-PRPS1/2/1L1 antibody


Cat. No.    JK160916     
Package    25 μl/100 μl/200 μl
Storage    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Product overview
Description                   Anti-PRPS1/2/1L1 rabbit polyclonal antibody
Applications        ELISA, WB, IHC 
Immunogen               Synthetic peptide of human PRPS1/2/1L1
Reactivity                 Human, Mouse
Content                     0.8 mg/ml
Host species               Rabbit
Ig class                  Immunogen-specific rabbit IgG
Purification                 Antigen affinity purification

Target information
Symbol                 PRPS1/2/1L1
Full name                 phosphoribosyl pyrophosphate synthetase 1/2/1-like 1
Synonyms    ARTS; DFN2; PRSI; CMTX5; DFNX1; PRS-I; PPRibP/PRSII/PRPS1; PRPS3; PRPSL; PRS-III
Swissprot    P60891/P11908/P21108

Target Background
PRPS (phosphoribosyl pyrophosphate synthetase) proteins catalyze the synthesis of phosphoribosyl pyrophosphate (PRPP). Three human PRPS isoforms exist and are encoded by three different genes. PRPS1 and PRPS2 (also known as PRS1 and PRS2, respectively) are ubiquitously expressed, while PRPS3 (also known as PRPS1L1) is specific to the testis. PRPP is an important substrate synthesized from MgATP and ribose-5-phosphate in a reaction that requires inorganic phosphate and magnesium as a cofactor. PRPP is essential in the synthesis of nearly all nucleotides, implying that PRPS1/2 play an important role in nucleotide biosynthesis and purine metabolism. A mutation in the gene encoding 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).