PRPS1/2 抗体 (EE-17): sc-100822

PRPS1/2 Antibody (EE-17) is an IgG₃ mouse monoclonal PRPS1/2 antibody (also designated PRPS1/2 antibody) that detects the PRPS1/2 protein of mouse, rat and human origin by WB and IP. PRPS1/2 Antibody (EE-17) is available as the non-conjugated anti-PRPS1/2 antibody. 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).

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References:

1. Accelerated transcription of PRPS1 in X-linked overactivity of normal human phosphoribosylpyrophosphate synthetase.  |  Ahmed, M., et al. 1999. J Biol Chem. 274: 7482-8. PMID: 10066814
2. Phosphoribosylpyrophosphate synthetase overactivity as a cause of uric acid overproduction in a young woman.  |  García-Pavía, P., et al. 2003. Arthritis Rheum. 48: 2036-41. PMID: 12847698
3. Promoter regions of the human X-linked housekeeping genes PRPS1 and PRPS2 encoding phosphoribosylpyrophosphate synthetase subunit I and II isoforms.  |  Ishizuka, T., et al. 1992. Biochim Biophys Acta. 1130: 139-48. PMID: 1314091
4. Phosphoribosyl pyrophosphate formation in the rat adrenal gland in relation to adrenal growth in experimental diabetes.  |  Kunjara, S., et al. 1992. Diabetes. 41: 1429-35. PMID: 1383069
5. Expression, purification, crystallization and preliminary X-ray diffraction analysis of human phosphoribosyl pyrophosphate synthetase 1 (PRS1).  |  Tang, W., et al. 2006. Acta Crystallogr Sect F Struct Biol Cryst Commun. 62: 432-4. PMID: 16682768
6. Crystal structure of human phosphoribosylpyrophosphate synthetase 1 reveals a novel allosteric site.  |  Li, S., et al. 2007. Biochem J. 401: 39-47. PMID: 16939420
7. Arts syndrome is caused by loss-of-function mutations in PRPS1.  |  de Brouwer, AP., et al. 2007. Am J Hum Genet. 81: 507-18. PMID: 17701896
8. Mutations in PRPS1, which encodes the phosphoribosyl pyrophosphate synthetase enzyme critical for nucleotide biosynthesis, cause hereditary peripheral neuropathy with hearing loss and optic neuropathy (cmtx5).  |  Kim, HJ., et al. 2007. Am J Hum Genet. 81: 552-8. PMID: 17701900
9. [PRPP synthetase superactivity].  |  Fujimori, S. 1996. Nihon Rinsho. 54: 3309-14. PMID: 8976111