Rabbit PDSS2 Polyclonal Antibody | anti-PDSS2 antibody

PDSS2 Rabbit Polyclonal

Cat. Num. AAA7602525

• Gene Names: PDSS2; DLP1; hDLP1; COQ10D3; C6orf210; bA59I9.3
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: PDSS2; Polyclonal Antibody; PDSS2 Rabbit Polyclonal; anti-PDSS2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 174

• Applicable Applications for anti-PDSS2 antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Immunogen: Prenyl (decaprenyl) diphosphate synthase, subunit 2
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human kidney using MBS7602525 (PDSS2 antibody) at dilution of 1:50)

Western Blot (WB)

(SGC-7901 cells were subjected to SDS PAGE followed by western blot with MBS7602525 (PDSS2 antibody) at dilution of 1:300)

NCBI and Uniprot Product Information

• NCBI GI #: 71051966
• NCBI GeneID: 57107
• Molecular Weight: 26,377 Da
• NCBI Official Full Name: PDSS2 protein
• NCBI Official Synonym Full Names: prenyl (decaprenyl) diphosphate synthase, subunit 2
• NCBI Official Symbol: PDSS2
• NCBI Official Synonym Symbols: DLP1; hDLP1; COQ10D3; C6orf210; bA59I9.3
• NCBI Protein Information: decaprenyl-diphosphate synthase subunit 2
• UniProt Protein Name: Decaprenyl-diphosphate synthase subunit 2
• Protein Family: Decaprenyl-diphosphate synthase
• UniProt Gene Name: PDSS2
• UniProt Synonym Gene Names: C6orf210; DLP1
• UniProt Entry Name: DLP1_HUMAN

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: EC 2.5.1.91; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Transferase

Chromosomal Location of Human Ortholog: 6q21

Cellular Component: cytoplasm; mitochondrial matrix

Molecular Function: protein heterodimerization activity; trans-hexaprenyltranstransferase activity; trans-octaprenyltranstransferase activity

Biological Process: isoprenoid biosynthetic process; protein heterotetramerization; regulation of body fluid levels; ubiquinone biosynthetic process

Disease: Coenzyme Q10 Deficiency, Primary, 3