COX10 Rabbit Polyclonal Antibody

COX10 Rabbit Polyclonal Antibody

Size1:50μl Price1:$118
Size2:100μl Price2:$220
Size3:500μl Price3:$980
SKU: APRab09265 Category: Polyclonal Antibody Tags: , , ,

Datasheet

Summary

Production Name

COX10 Rabbit Polyclonal Antibody

Description

Rabbit Polyclonal Antibody

Host

Rabbit

Application

WB,ELISA

Reactivity

Human,Rat,Mouse

 

Performance

Conjugation

Unconjugated

Modification

Unmodified

Isotype

IgG

Clonality

Polyclonal

Form

Liquid

Storage

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.

Buffer

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

Purification

Affinity purification

 

Immunogen

Gene Name

COX10

Alternative Names

COX10; Protoheme IX farnesyltransferase; mitochondrial; Heme O synthase

Gene ID

1352

SwissProt ID

Q12887

 

Application

Dilution Ratio

WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.

Molecular Weight

49kD

 

Background

Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes heme A:farnesyltransferase, which is not a structural subunit but required for the expression of functional COX and functions in the maturation of the heme A prosthetic group of COX. This protein is predicted to contain 7-9 transmembrane domains localized in the mitochondrial inner membrane. A gene mutation, which results in the substitution of a lysdisease:Defects in COX10 are a cause of cytochrome c oxidase deficiency (COX deficiency) [MIM:220110]. COX deficiency is a clinically heterogeneous disorder. The clinical features are ranging from isolated myopathy to severe multisystem disease, with onset from infancy to adulthood.,disease:Defects in COX10 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.,function:Converts protoheme IX and farnesyl diphosphate to heme O.,similarity:Belongs to the ubiA prenyltransferase family.,

 

Research Area

Oxidative phosphorylation;Porphyrin and chlorophyll metabolism;