Rabbit Polyclonal Antibody to PRPS1/2/1L1

包装: 1G|100G|500G|1KG|100KG

现货支持定制

QQ咨询
| 微信咨询

满200元免运费
正品保障提供发票
现货闪电当时发货

别名:ARTS; DFN2; PRSI; CMTX5; DFNX1; PRS-I; PPRibP/PRSII/PRPS1; PRPS3; PRPSL; PRS-III
应用:WB,IHC
反应种属:Human, Mouse
规格：50μl/100μl

Description
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).

Description

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).

Specification
Aliases	ARTS; DFN2; PRSI; CMTX5; DFNX1; PRS-I; PPRibP/PRSII/PRPS1; PRPS3; PRPSL; PRS-III
Swissprot	P60891/P11908/P21108
WB Predicted band size	35 kDa
Host/Isotype	Rabbit IgG
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity	Human, Mouse
Immunogen	Synthetic peptide of human PRPS1/2/1L1
Formulation	pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Application
WB	1/500-1/2000
IHC	1/25-1/100
ELISA	1/2000-1/5000

	Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: 293T cells, Primary antibody: P11215(PRPS1/2/1L1 Antibody) at dilution 1/400, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 1 minute
	The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using P11215(PRPS1/2/1L1 Antibody) at dilution 1/30. (Original magnification: ×200)