Key features and details
- Rabbit polyclonal to PRPS1
- Suitable for: IHC-P, ICC/IF
- Reacts with: Human
- Isotype: IgG
Product nameAnti-PRPS1 antibody
See all PRPS1 primary antibodies
DescriptionRabbit polyclonal to PRPS1
Tested applicationsSuitable for: IHC-P, ICC/IFmore details
Species reactivityReacts with: Human
- IHC-P: Human endometrium and lymph node tissue. ICC/IF: U-251 MG cells.
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab247049 in the following tested applications.
|IHC-P||1/20 - 1/50. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
|ICC/IF||Use a concentration of 0.25 - 2 µg/ml.
Fixation/Permeabilization: PFA/Triton X-100.
FunctionCatalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.
PathwayMetabolic intermediate biosynthesis; 5-phospho-alpha-D-ribose 1-diphosphate biosynthesis; 5-phospho-alpha-D-ribose 1-diphosphate from D-ribose 5-phosphate (route I): step 1/1.
Involvement in diseaseDefects in PRPS1 are the cause of phosphoribosylpyrophosphate synthetase superactivity (PRPS1 superactivity) [MIM:300661]; 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) [MIM:311070]; 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) [MIM:301835]; 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) [MIM:304500]; 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.
Sequence similaritiesBelongs to the ribose-phosphate pyrophosphokinase family.
- Information by UniProt
- ARTS antibody
- CMTX5 antibody
- Deafness 2 perceptive congenital antibody
PFA fixed, Triton X-100 permeabilized U-251 MG (Human brain glioma cell line) cells labeling PRPS1 using ab247049 at 4 µg/ml (green) in ICC/IF.
Paraffin-embedded human lymph node tissue stained for PRPS1 using ab247049 at 1/20 dilution in immunohistochemical analysis.
Paraffin-embedded human endometrium tissue stained for PRPS1 using ab247049 at 1/20 dilution in immunohistochemical analysis.
Paraffin-embedded human pancreas tissue stained for PRPS1 using ab247049 at 1/20 dilution in immunohistochemical analysis. Low positivity in exocrine glandular cells.
Paraffin-embedded human prostate tissue stained for PRPS1 using ab247049 at 1/20 dilution in immunohistochemical analysis.
ab247049 has not yet been referenced specifically in any publications.