E-AB-15944
PRPS1/2/PRPS1L1 Polyclonal Antibody
Größe
£106.20
War £118.00
- SKU:
- E-AB-15944
- Zusätzliche Namen:
- ARTS,CMTX5,DFN2,DFNX1,PPRibP/PRSII/PRPS1,PRPS3,PRPSL,PRS-I,PRS-III,PRSI
- Anwendung:
- IHC, WB
- Konzentration:
- 0.4 mg/ml
- Aufreinigung:
- Affinity Purified
- Lagerbedingungen:
- -20[o]C Avoid freeze/thaw cycles.
- Hersteller:
- Elabscience
- Host:
- Rabbit
- Reaktivitäten:
- Human, Mouse
- Buffer:
- Phosphate buffered solution, pH 7.4, containing 0.05% stabilizer and 50% glycerol.
- Immunogen:
- Synthetic peptide of human PRPS1/2/1L1
- Uniprot:
- P11908, P21108, P60891
- Synonyme:
- ARTS;CMTX5;deafness 2, perceptive, congenital;deafness, X-linked 2, perceptive, congenital;DFN2;DFNX1;dJ1070B1.2 (phosphoribosyl pyrophosphate synthetase 1);phosphoribosyl pyrophosphate synthase 1-like 1;phosphoribosyl pyrophosphate synthase I;phosphoribosyl pyrophosphate synthase II;phosphoribosyl pyrophosphate synthase III;phosphoribosylpyrophosphate synthetase subunit III;PPRibP;PPRibP synthetase;PRPS1;PRPS1-like 1;PRPS3;PRPSL;PRS-I;PRS-II;PRS-III;PRSI;PRSII;ribose-phosphate diphosphokinase 1;ribose-phosphate diphosphokinase 2;ribose-phosphate diphosphokinase catalytic chain III;ribose-phosphate pyrophosphokinase 1;ribose-phosphate pyrophosphokinase 2;ribose-phosphate pyrophosphokinase 3;ribose-phosphate pyrophosphokinase III;testicular secretory protein Li 41
- Weitere Details:
- 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).
- Versandbedingungen:
- Blue Ice