Rabbit anti-Human, Mouse PDSS1 Polyclonal Antibody | anti-PDSS1 antibody
PDSS1 antibody
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene is an enzyme that elongates the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. The gene product catalyzes the formation of all trans-polyprenyl pyrophosphates from isopentyl diphosphate in the assembly of polyisoprenoid side chains, the first step in coenzyme Q biosynthesis. The protein may be peripherally associated with the inner mitochondrial membrane, though no transit peptide has been definitively identified to date. Defects in this gene are a cause of coenzyme Q10 deficiency. [provided by RefSeq, Jul 2008]
Uniprot Description
DPS1: Supplies decaprenyl diphosphate, the precursor for the side chain of the isoprenoid quinones ubiquinone-10. Defects in PDSS1 are the cause of coenzyme Q10 deficiency, primary, type 2 (COQ10D2). An autosomal recessive multisystem disorder characterized by early-onset deafness, optic atrophy, mild mental retardation, peripheral neuropathy, obesity, livedo reticularis, and cardiac valvulopathy. Belongs to the FPP/GGPP synthase family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; EC 2.5.1.91; Transferase
Chromosomal Location of Human Ortholog: 10p12.1
Cellular Component: mitochondrial matrix
Molecular Function: protein heterodimerization activity; metal ion binding; trans-hexaprenyltranstransferase activity; trans-octaprenyltranstransferase activity
Biological Process: ubiquinone biosynthetic process; isoprenoid biosynthetic process; protein heterotetramerization
Disease: Coenzyme Q10 Deficiency, Primary, 2
Research Articles on PDSS1
Similar Products
Product Notes
The PDSS1 pdss1 (Catalog #AAA5300712) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The PDSS1 antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's PDSS1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 5 ug/ml. Researchers should empirically determine the suitability of the PDSS1 pdss1 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 "PDSS1, 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
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
