PDSS2 Rabbit pAb
- 产品详情
- 实验流程
Application
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | Q86YH6 |
| Other Accession | Q86YH6 |
| Reactivity | Human |
| Predicted | Rat, Mouse |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 44129 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human PDSS2 |
| Epitope Specificity | 21-100/399 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the FPP/GGPP synthase family. |
| SUBUNIT | Heterotetramer of 2 DPS1/TPRT and 2 DLP1 subunits. |
| DISEASE | Defects in PDSS2 are the cause of coenzyme Q10 deficiency, primary, type 3 (COQ10D3) [MIM:614652]. A fatal encephalomyopathic form of coenzyme Q10 deficiency with nephritic 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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. |
| Gene ID | 57107 |
|---|---|
| Other Names | C6orf210; COQ10D3; COQ1B; DLP1; bA59I9.3; hDLP1; 5430420P03Rik; Gm60; Plmp; kd; mDLP1; DLP1_HUMAN; PDSS2; All-trans-decaprenyl-diphosphate synthase subunit 2; Candidate tumor suppressor protein; Decaprenyl pyrophosphate synthase subunit 2; Decaprenyl-diphosphate synthase subunit 2; Solanesyl-diphosphate synthase subunit 2; DLP1_MOUSE; DLP1_RAT; decaprenyl diphosphate synthase subunit 2; chromosome 6 open reading frame 210; prenyl (decaprenyl) diphosphate synthase, subunit 2 |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | PDSS2 (HGNC:23041) |
|---|---|
| Synonyms | C6orf210, DLP1 |
| Function | Heterotetrameric enzyme that catalyzes the condensation of farnesyl diphosphate (FPP), which acts as a primer, and isopentenyl diphosphate (IPP) to produce prenyl diphosphates of varying chain lengths and participates in the determination of the side chain of ubiquinone (PubMed:16262699). Supplies nona and decaprenyl diphosphate, the precursors for the side chain of the isoprenoid quinones ubiquinone-9 (Q9) and ubiquinone-10 (Q10) respectively (PubMed:16262699). The enzyme adds isopentenyl diphosphate molecules sequentially to farnesyl diphosphate with trans stereochemistry (PubMed:16262699). May play a role during cerebellar development (By similarity). May regulate mitochondrial respiratory chain function (By similarity). |
| Cellular Location | Mitochondrion. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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