Rabbit Anti-DPS1/FITC Conjugated antibody

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Coenzyme Q1 homolog; COQ1; Decaprenyl pyrophosphate synthetase subunit 1; DPS; DPS1_HUMAN; hDPS1; Polyprenyl pyrophosphate synthetase; Prenyl (decaprenyl) diphosphate synthase, subunit 1; SPS; TPRT; TPT; Trans prenyltransferase.

Cat:

SL14422R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Pig,Cow,Horse,Rabbit,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human DPS1

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

46kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
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]

Function:
DPS1 is an enzyme that elongates the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. It 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. There are three named isoforms.

Subunit:
Heterotetramer of 2 DPS1/TPRT and 2 DLP1 subunits.

Subcellular Location:
Mitochondrial and Plasma membrane

DISEASE:
Coenzyme Q10 deficiency, primary, 2 (COQ10D2) [MIM:614651]: An autosomal recessive multisystem disorder characterized by early-onset deafness, optic atrophy, mild mental retardation, peripheral neuropathy, obesity, livedo reticularis, and cardiac valvulopathy. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the FPP/GGPP synthase family.

Database links:

Entrez Gene: 23590 Human

Entrez Gene: 56075 Mouse

Omim: 607429 Human

SwissProt: Q5T2R2 Human

SwissProt: Q33DR2 Mouse

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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