Immunohistochemistry (IHC)
(Immunohistochemical analysis of PDSS2 protein in paraffin embedded Human lymph node tissue using PDSS2 antibody)
Mouse PDSS2 Monoclonal Antibody | anti-PDSS2 antibody
PDSS2 antibody
WB: 1:2000
Immunohistochemistry (IHC)
(Immunohistochemical analysis of PDSS2 protein in paraffin embedded Human lymph node tissue using PDSS2 antibody)
Immunohistochemistry (IHC)
(Immunohistochemical analysis of PDSS2 protein in paraffin embedded Human lymph node tissue using PDSS2 antibody)
Western Blot (WB)
(Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant PDSS2 protein (Right) or empty vector (Left) detected with PDSS2 antibody)
Western Blot (WB)
(Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant PDSS2 protein (Right) or empty vector (Left) detected with PDSS2 antibody)
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene is an enzyme that synthesizes 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. Defects in this gene are a cause of coenzyme Q10 deficiency.[provided by RefSeq, Oct 2009]
Uniprot Description
PDSS2: Supplies decaprenyl diphosphate, the precursor for the side chain of the isoprenoid quinones ubiquinone-10. Defects in PDSS2 are the cause of coenzyme Q10 deficiency, primary, type 3 (COQ10D3). A fatal encephalomyopathic form of coenzyme Q10 deficiency with nephrotic syndrome. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy; Leigh syndrome with growth retardation; and an isolated myopathic form. Belongs to the FPP/GGPP synthase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Transferase; EC 2.5.1.91
Chromosomal Location of Human Ortholog: 6q21
Cellular Component: mitochondrial matrix; cytoplasm
Molecular Function: protein heterodimerization activity; trans-hexaprenyltranstransferase activity; trans-octaprenyltranstransferase activity
Biological Process: ubiquinone biosynthetic process; regulation of body fluid levels; isoprenoid biosynthetic process; protein heterotetramerization
Disease: Coenzyme Q10 Deficiency, Primary, 3
Research Articles on PDSS2
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Product Notes
The PDSS2 pdss2 (Catalog #AAA834399) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's PDSS2 can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC), Western Blot (WB). IHC: 1:150 WB: 1:2000. Researchers should empirically determine the suitability of the PDSS2 pdss2 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 "PDSS2, Monoclonal 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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