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Rabbit Anti-PRPS1 Antibody (CSB-PA018776ESR2HU)
Supplier: CUSABIO BIOTECH CO.
Ribose-phosphate pyrophosphokinase 1 Antibody,PPRibP Antibody,Phosphoribosyl pyrophosphate synthase I Antibody,PRS-I Antibody,PRPS1 AntibodyMore alternative names for the antibody
ARTS antibody|CMTX5 antibody|Deafness 2 perceptive congenital antibody|Deafness X linked 2 perceptive congenital antibody|DFN2 antibody|DFNX1 antibody|EC 126.96.36.199 antibody|KIAA0967 antibody|Phosphoribosyl pyrophosphate synthase I antibody|Phosphoribosyl pyrophosphate synthetase I antibody|PPRibP antibody|Prps1 antibody|PRPS1_HUMAN antibody|PRS I antibody|PRS-I antibody|PRSI antibody|Ribose phosphate pyrophosphokinase I antibody|Ribose-phosphate pyrophosphokinase 1 antibody
Anti-PRPS1 antibody (ab137577)
Anti-PRPS1 antibody (ab137577)
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Ribose-phosphate pyrophosphokinase 1 protein (1-318AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Database links|| |
Entrez Gene:781227( Cow), Entrez Gene:5631( Human), Entrez Gene:19139( Mouse), Entrez Gene:29562( Rat), Entrez Gene:314140( Rat), Omim:311850( Human), SwissProt:Q2HJ58( Cow), SwissProt:P60891( Human), SwissProt:Q9D7G0( Mouse), SwissProt:P60892( Rat), Unigene:56( Human), Unigene:287178( Mouse), Unigene:91470( Rat), Unigene:9761( Rat)
|Protein function|| |
Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.
|Involvement in disease|| |
Note=Phosphoribosyl pyrophosphate synthetase I deficiency is a rare condition caused by mutations in PRPS1 that lead to variable disease phenotypes including optic atrophy, retinitis pigmentosa, ataxia, peripheral neuropathy and hearing loss. .; Phosphoribosylpyrophosphate synthetase superactivity (PRPS1 superactivity) [MIM:300661]: Familial disorder characterized by excessive purine production, gout and uric acid urolithiasis. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Charcot-Marie-Tooth disease, X-linked recessive, 5 (CMTX5) [MIM:311070]: A form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. 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. . Note=The disease is caused by mutations affecting the gene represented in this entry.; ARTS syndrome (ARTS) [MIM:301835]: 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. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Deafness, X-linked, 1 (DFNX1) [MIM:304500]: A form of deafness characterized by progressive, severe-to-profound sensorineural hearing loss in males. Females manifest mild to moderate hearing loss. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the ribose-phosphate pyrophosphokinase family.
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CUSABIO BIOTECH CO.
|Product type|| |
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