Rabbit anti-Human, Mouse PDSS1 Polyclonal Antibody | anti-PDSS1 antibody

PDSS1 antibody

Cat. Num. AAA5300712

• Gene Names: PDSS1; DPS; SPS; TPT; COQ1; TPRT; TPT 1; hDPS1; COQ10D2
• Reactivity: Human, Mouse
• Applications: Western Blot
• Purity: Total IgG Protein A purified
• Synonyms: PDSS1; Polyclonal Antibody; PDSS1 antibody; Polyclonal PDSS1; Anti-PDSS1; TPT; Prenyl decaprenyl diphosphate synthase subunit 1; TPRT; COQ1; PDSS 1; PDSS-1; hDPS1; MGC70953; Decaprenyl Diphosphate Synthase Subunit 1; RP13-16H11.3; anti-PDSS1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Purity/Purification: Total IgG Protein A purified
• Form/Format: Lyophilized powder. Add 100ul distilled water for a 1mg/ml concentration of PDSS1 antibody in PBS
• Concentration: 1 mg/ml (varies by lot)
• Sequence Length: 306

• Applicable Applications for anti-PDSS1 antibody: Western Blot (WB)
• Application Notes: WB: 5 ug/ml
• Biological Significance: PDSS1 is an enzyme that elongates the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. PDSS1 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 PDSS1 gene are a cause of coenzyme Q10 deficiency.
• Cross-Reactivity: Human,Mouse
• Immunogen: PDSS1 antibody was raised using a synthetic peptide corresponding to a region with amino acids GEFLQLGSKENENERFAHYLEKTFKKTASLIANSCKAVSVLGCPDPVVHE
• Preparation and Storage: Store at 2-8 degree C for short periods. For longer periods of storage, store at -20 degree C. Avoid repeat freeze-thaw cycles.

Related Product Information for anti-PDSS1 antibody

Rabbit polyclonal PDSS1 antibody

Product Categories/Family for anti-PDSS1 antibody

Cell Biology; Purified Polyclonal Antibodies

NCBI and Uniprot Product Information

• NCBI GI #: 39645583
• NCBI GeneID: 23590
• Molecular Weight: 46 kDa (MW of target protein)
• NCBI Official Full Name: PDSS1 protein
• NCBI Official Synonym Full Names: prenyl (decaprenyl) diphosphate synthase, subunit 1
• NCBI Official Symbol: PDSS1
• NCBI Official Synonym Symbols: DPS; SPS; TPT; COQ1; TPRT; TPT 1; hDPS1; COQ10D2
• NCBI Protein Information: decaprenyl-diphosphate synthase subunit 1
• UniProt Protein Name: Decaprenyl-diphosphate synthase subunit 1
• UniProt Gene Name: PDSS1
• UniProt Synonym Gene Names: DPS1; TPRT; TPT 1
• UniProt Entry Name: DPS1_HUMAN

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