Ribose-Phosphate Pyrophosphokinase 1 (PRPS1), ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of PRPS1 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9366452 is a ready-to-use microwell, strip-or-full plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Ribose-Phosphate Pyrophosphokinase 1 (PRPS1) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing PRPS1. The ELISA analytical biochemical technique of the MBS9366452 kit is based on PRPS1 antibody-PRPS1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect PRPS1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, PRPS1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

27,526 Da

phosphoribosyl pyrophosphate synthetase 1

ribose-phosphate pyrophosphokinase 1

Ribose-phosphate pyrophosphokinase 1

PRS-I  [Similar Products]

PRPS1_HUMAN

This gene encodes an enzyme that catalyzes the phosphoribosylation of ribose 5-phosphate to 5-phosphoribosyl-1-pyrophosphate, which is necessary for purine metabolism and nucleotide biosynthesis. Defects in this gene are a cause of phosphoribosylpyrophosphate synthetase superactivity, Charcot-Marie-Tooth disease X-linked recessive type 5 and Arts Syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Feb 2011]

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: Nucleotide Metabolism - purine; EC 2.7.6.1; Carbohydrate Metabolism - pentose phosphate pathway; Kinase, other

Chromosomal Location of Human Ortholog: Xq22.3

Cellular Component: cytosol

Molecular Function: ADP binding; AMP binding; ATP binding; carbohydrate binding; GDP binding; kinase activity; magnesium ion binding; protein binding; protein homodimerization activity; ribose phosphate diphosphokinase activity

Biological Process: 5-phosphoribose 1-diphosphate biosynthetic process; AMP biosynthetic process; carbohydrate metabolic process; hypoxanthine biosynthetic process; nervous system development; organ regeneration; phosphorylation; purine base metabolic process; purine nucleotide biosynthetic process; pyrimidine nucleotide biosynthetic process

Disease: Arts Syndrome; Charcot-marie-tooth Disease, X-linked Recessive, 5; Phosphoribosylpyrophosphate Synthetase Superactivity

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