COA4 Human

Cytochrome C Oxidase Assembly Factor 4 Human Recombinant
Cat. No.
BT893
Source
Escherichia Coli.
Synonyms
CHCHD8, CMC3, E2IG2, Cytochrome c oxidase assembly factor 4 homolog, mitochondrial, Coiled-coil-helix-coiled-coil-helix domain-containing protein 8, E2-induced gene 2 protein, COA4.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 90.0% 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.
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Description

COA4 Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 110 amino acids (1-87 a.a) and having a molecular mass of 12.5kDa.
COA4 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction
Cytochrome c oxidase assembly factor 4 (COA4) is essential for the assembly and function of cytochrome c oxidase (COX), the terminal enzyme in the mitochondrial respiratory chain. COX plays a crucial role in oxidative phosphorylation, a process that generates energy in cells. COA4 is required for the formation of COX, which is organized into macromolecular supercomplexes within mitochondria. Defects in the COX4 gene, which encodes COA4, can lead to severe neurological disorders known as encephalomyopathies.
Description
This product consists of the recombinant human COA4 protein, produced in E. coli. It is a single polypeptide chain that is not glycosylated and contains 110 amino acids, spanning from position 1 to 87 of the native protein. The protein has a molecular weight of 12.5 kDa. For purification and detection purposes, a 23 amino acid His-tag is added to the N-terminus of the protein. The purification process involves proprietary chromatographic techniques to ensure high purity.
Physical Appearance
The product is a sterile, colorless solution that has been filtered for sterility.
Formulation
The COA4 protein is supplied in a solution containing 0.25 mg/ml of the protein in a buffer consisting of 20 mM Tris-HCl (pH 8.0), 0.15 M NaCl, 10% glycerol, and 1 mM DTT. This formulation helps to maintain the stability and activity of the protein.
Stability
For short-term storage (up to 2-4 weeks), the product can be stored at 4°C. For longer storage, it is recommended to store the protein at -20°C. To further enhance long-term stability, adding a carrier protein such as 0.1% HSA or BSA is advised. To prevent protein degradation, avoid repeated freezing and thawing of the product.
Purity
The purity of the COA4 protein is greater than 90%, as determined by SDS-PAGE analysis, indicating a high level of purity.
Synonyms
CHCHD8, CMC3, E2IG2, Cytochrome c oxidase assembly factor 4 homolog, mitochondrial, Coiled-coil-helix-coiled-coil-helix domain-containing protein 8, E2-induced gene 2 protein, COA4.
Source
Escherichia Coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MGSMSTSVPQ GHTWTQRVKK DDEEEDPLDQ LISRSGCAAS HFAVQECMAQ HQDWRQCQPQ VQAFKDCMSE QQARRQEELQ RRQEQAGAHH.

Product Science Overview

Structure and Function

Cytochrome c oxidase is a large transmembrane protein complex found in the mitochondria of eukaryotes. It is composed of multiple subunits, including two hemes (cytochrome a and cytochrome a3) and two copper centers (CuA and CuB) . The enzyme facilitates the transfer of electrons from cytochrome c to molecular oxygen, ultimately producing water and contributing to the proton gradient used by ATP synthase to generate ATP .

COA4 is involved in the proper assembly and stabilization of this complex. It ensures that the various subunits and cofactors are correctly positioned and functional . This assembly process is vital for the efficient functioning of the electron transport chain and, consequently, for cellular respiration and energy production .

Genetic and Clinical Relevance

Mutations or deficiencies in COA4 can lead to various mitochondrial disorders. For instance, COA4 has been associated with Bardet-Biedl Syndrome 18 and Spinal Cord Primitive Neuroectodermal Neoplasm . These conditions highlight the importance of COA4 in maintaining mitochondrial function and overall cellular health.

Research and Applications

Human recombinant COA4 is used in research to study its role in mitochondrial function and its potential implications in various diseases. By understanding how COA4 contributes to the assembly of cytochrome c oxidase, scientists can develop targeted therapies for mitochondrial disorders and improve our understanding of cellular energy metabolism .

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THE BIOTEK
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