catalog number :
MBS9325225
products type :
ELISA Kit
products full name :
Mouse Ribose-phosphate pyrophosphokinase 1, PRPS1 ELISA Kit
products short name :
phosphoribosyl pyrophosphate synthetase 1
products name syn :
Mouse Ribose-phosphate pyrophosphokinase 1 (PRPS1) ELISA kit; RP11-540N4.1; ARTS; CMTX5; KIAA0967; PPRibP; PRSI; dJ1070B1.2 (phosphoribosyl pyrophosphate synthetase 1) ; phosphoribosyl pyrophosphate synthetase 1
other names :
ribose-phosphate pyrophosphokinase 1; Ribose-phosphate pyrophosphokinase 1; ribose-phosphate pyrophosphokinase 1; phosphoribosyl pyrophosphate synthase I; phosphoribosyl pyrophosphate synthetase I; phosphoribosyl pyrophosphate synthetase 1; Phosphoribosyl pyrophosphate synthase I; PRS-I
products gene name :
PRPS1
other gene names :
Prps1; Prps1; PRS-I; C76571; C76678; Prps-1; 2310010D17Rik; PRS-I
uniprot entry name :
PRPS1_MOUSE
specificity :
No significant cross-reactivity or interference between this analyte and analogues is observed.
storage stability :
Store all reagents at 2-8 degree C
other info1 :
Samples: Serum, Plasma and Tissue Homogenate. Assay Type: Sandwich. Detection Range: 0.5 ng/ml - 16 ng/ml. Sensitivity: 0.1 ng/ml.
other info2 :
Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].
products description :
Background: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of PRPS1 (hereafter termed this analyte) in undiluted original Mouse serum, plasma and tissue homogenate samples.
ncbi acc num :
NP_067438.1
ncbi gb acc num :
NM_021463.4
ncbi mol weight :
34,834 Da
ncbi pathways :
5-Phosphoribose 1-diphosphate Biosynthesis Pathway (574753); Metabolism Pathway (574739); Metabolism Of Carbohydrates Pathway (574740); Nucleotide Metabolism Pathway (198341); PRPP Biosynthesis, Ribose 5P = PRPP Pathway (421744); PRPP Biosynthesis, Ribose 5P = PRPP Pathway (468198); Pentose Phosphate Pathway (83128); Pentose Phosphate Pathway (289); Purine Metabolism Pathway (83144); Purine Metabolism Pathway (307)
uniprot summary :
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: EC 2.7.6.1; Carbohydrate Metabolism - pentose phosphate pathway; Nucleotide Metabolism - purine; Kinase, other. Cellular Component: protein complex. Molecular Function: ribose phosphate diphosphokinase activity; transferase activity; protein homodimerization activity; GDP binding; metal ion binding; nucleotide binding; magnesium ion binding; kinase activity; carbohydrate binding; ADP binding; ATP binding; AMP binding. Biological Process: AMP biosynthetic process; cellular biosynthetic process; nervous system development; ribose phosphate metabolic process; nucleotide biosynthetic process; hypoxanthine biosynthetic process; ribonucleoside monophosphate biosynthetic process; 5-phosphoribose 1-diphosphate biosynthetic process; purine nucleotide biosynthetic process; phosphorylation; purine base metabolic process