Rabbit PAPSS2 Polyclonal Antibody | anti-PAPSS2 antibody
PAPSS2 Rabbit Polyclonal
Immunogen Affinity Purified
Immunogen Affinity Purified
NCBI and Uniprot Product Information
NCBI Description
Sulfation is a common modification of endogenous (lipids, proteins, and carbohydrates) and exogenous (xenobiotics and drugs) compounds. In mammals, the sulfate source is 3'-phosphoadenosine 5'-phosphosulfate (PAPS), created from ATP and inorganic sulfate. Two different tissue isoforms encoded by different genes synthesize PAPS. This gene encodes one of the two PAPS synthetases. Defects in this gene cause the Pakistani type of spondyloepimetaphyseal dysplasia. Two alternatively spliced transcript variants that encode different isoforms have been described for this gene. [provided by RefSeq, Jul 2008]
Uniprot Description
PAPSS2: Bifunctional enzyme with both ATP sulfurylase and APS kinase activity, which mediates two steps in the sulfate activation pathway. The first step is the transfer of a sulfate group to ATP to yield adenosine 5'-phosphosulfate (APS), and the second step is the transfer of a phosphate group from ATP to APS yielding 3'-phosphoadenylylsulfate (PAPS: activated sulfate donor used by sulfotransferase). In mammals, PAPS is the sole source of sulfate; APS appears to be only an intermediate in the sulfate- activation pathway. May have a important role in skeletogenesis during postnatal growth. Defects in PAPSS2 are the cause of spondyloepimetaphyseal dysplasia Pakistani type (SEMD-PA). A bone disease characterized by epiphyseal dysplasia with mild metaphyseal abnormalities. Clinical features include short stature evidenced at birth, short and bowed lower limbs, mild brachydactyly, kyphoscoliosis, abnormal gait, enlarged knee joints. Some patients may manifest premature pubarche and hyperandrogenism associated with skeletal dysplasia and short stature. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Transferase; Nucleotide Metabolism - purine; Energy Metabolism - sulfur; EC 2.7.1.25; EC 2.7.7.4; Other Amino Acids Metabolism - selenoamino acid
Chromosomal Location of Human Ortholog: 10q24
Cellular Component: cytosol
Molecular Function: adenylylsulfate kinase activity; ATP binding; nucleotidyltransferase activity; sulfate adenylyltransferase (ATP) activity
Biological Process: 3'-phosphoadenosine 5'-phosphosulfate biosynthetic process; blood coagulation; phosphorylation; skeletal development; sulfate assimilation
Disease: Brachyolmia Type 4 With Mild Epiphyseal And Metaphyseal Changes
Research Articles on PAPSS2
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Product Notes
The PAPSS2 papss2 (Catalog #AAA7602376) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PAPSS2 Rabbit Polyclonal reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's PAPSS2 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB). Researchers should empirically determine the suitability of the PAPSS2 papss2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "PAPSS2, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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