SCO2 Human

SCO Cytochrome Oxidase Deficient Homolog 2 Human Recombinant
Cat. No.
BT27140
Source
Escherichia Coli.
Synonyms
SCO1L, SCO Cytochrome Oxidase Deficient Homolog 2 (yeast), Protein SCO2 Homolog-Mitochondrial, MGC125823, MGC125825.
Appearance
Sterile Filtered clear solution.
Purity
Greater than 90% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
Shipped with Ice Packs
In Stock

Description

SCO2 produced in E.Coli is a single, non-glycosylated polypeptide chain containing 246 amino acids (42-266a.a.) and having a molecular mass of 27.4kDa.
SCO2 is fused to a 21 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction
SCO2 protein belongs to the SCO1/2 family. Located on the inner mitochondrial membrane, SCO1 and SCO2 proteins play a crucial role in copper insertion or transport to the active site of cytochrome c oxidase (COX). Defects in SCO2 lead to fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC), characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. This deficiency results in decreased COX activity in the heart and skeletal muscle, while the liver and fibroblasts exhibit mild COX deficiencies.
Description
Produced in E. coli, SCO2 is a single, non-glycosylated polypeptide chain comprising 246 amino acids (42-266a.a.) with a molecular weight of 27.4kDa. This protein is fused to a 21 amino acid His-tag at the N-terminus and purified using proprietary chromatographic techniques.
Physical Appearance
A clear solution that has been sterilized by filtration.
Formulation
The SCO2 protein solution (0.5mg/ml) is supplied in a buffer containing 20mM Tris-HCl (pH 8.0), 2mM DTT, 200mM NaCl, and 30% glycerol.
Purity
Purity exceeding 90% as determined by SDS-PAGE analysis.
Stability
For short-term storage (2-4 weeks), keep at 4°C. For extended storage, freeze at -20°C. Repeated freezing and thawing should be avoided.
Synonyms
SCO1L, SCO Cytochrome Oxidase Deficient Homolog 2 (yeast), Protein SCO2 Homolog-Mitochondrial, MGC125823, MGC125825.
Source
Escherichia Coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MGPAETGGQG QPQGPGLRTR LLITGLFGAG LGGAWLALRA EKERLQQQKR TEALRQAAVG QGDFHLLDHR GRARCKADFR GQWVLMYFGF THCPDICPDE LEKLVQVVRQ LEAEPGLPPV QPVFITVDPE RDDVEAMARY VQDFHPRLLG LTGSTKQVAQ ASHSYRVYYN AGPKDEDQDY IVDHSIAIYL LNPDGLFTDY YGRSRSAEQI SDSVRRHMAA FRSVLS

Product Science Overview

Introduction

SCO Cytochrome Oxidase Deficient Homolog 2, commonly referred to as SCO2, is a protein encoded by the SCO2 gene in humans. This protein plays a crucial role in the assembly and function of cytochrome c oxidase (COX), which is a key component of the mitochondrial respiratory chain. The recombinant form of this protein is produced using various expression systems for research and therapeutic purposes.

Gene and Protein Structure

The SCO2 gene is located on the q arm of chromosome 22 at position 13.33 and spans approximately 2,871 base pairs . The gene produces a 15.1 kDa protein composed of 136 amino acids . The protein contains an N-terminal mitochondrial targeting presequence of 41 amino acids, which is essential for its localization to the mitochondria .

Function

SCO2 is a metallochaperone involved in the biogenesis of cytochrome c oxidase subunit II (MT-CO2/COX2). It is essential for the synthesis and maturation of this subunit by transporting copper to the Cu(A) site on MT-CO2/COX2 . Additionally, SCO2 acts as a thiol-disulfide oxidoreductase, regulating the redox state of cysteines in SCO1 during the maturation of MT-CO2/COX2 .

Biological Significance

Cytochrome c oxidase (COX) catalyzes the transfer of electrons from cytochrome c to molecular oxygen, which helps maintain the proton gradient across the inner mitochondrial membrane. This gradient is necessary for aerobic ATP production . Therefore, SCO2 is vital for cellular energy production and overall mitochondrial function.

Associated Diseases

Mutations in the SCO2 gene are associated with several disorders, including fatal infantile encephalocardiomyopathy and myopia 6 . These conditions highlight the importance of SCO2 in normal cellular and physiological functions.

Recombinant SCO2

The recombinant form of SCO2 is produced using expression systems such as E. coli. This recombinant protein is used in various research applications to study its function, interactions, and role in diseases . The recombinant SCO2 protein is typically purified to high levels of purity and is available in lyophilized form for ease of use in laboratory settings .

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