Mouse Anti-PDSS2 Recombinant Antibody (1C11) (CBMAB-P1366-YC)

Name: Mouse Anti-PDSS2 Recombinant Antibody (1C11)
SKU: CBMAB-P1366-YC
Rating: 5 (1 reviews)

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Datasheet

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Basic Information

Host Animal

Mouse

Clone

1C11

Application

IHC, WB

Immunogen

Full length human recombinant protein of human PDSS2 produced in HEK293T cell

Specificity

Human

Antibody Isotype

IgG2a

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

PBS, pH 7.3, 1% BSA, 50% glycerol, 0.02% sodium azide

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

Decaprenyl Diphosphate Synthase Subunit 2

Introduction

PDSS2 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.

Entrez Gene ID

57107

UniProt ID

Q86YH6

Alternative Names

Decaprenyl Diphosphate Synthase Subunit 2; Prenyl (Decaprenyl) Diphosphate Synthase, Subunit 2; All-Trans-Decaprenyl-Diphosphate Synthase Subunit 2; Decaprenyl Pyrophosphate Synthase Subunit 2; C6orf210; DLP1; Decaprenyl Pyrophosphate Synthetase Subunit 2; Subunit 2 Of Decaprenyl Diphosphate Synthase;

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).

Biological Process

Cerebellum developmentISS:UniProtKB
Isoprenoid biosynthetic processManual Assertion Based On ExperimentIDA:HGNC-UCL
Regulation of body fluid levelsIEA:Ensembl
Ubiquinone biosynthetic processManual Assertion Based On ExperimentIDA:HGNC-UCL

Cellular Location

Mitochondrion

Involvement in disease

Coenzyme Q10 deficiency, primary, 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.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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