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ETHE1 Protein, Human, Recombinant (His Tag)

Product Specifications

Background

ETHE1, also known as HSCO, is a sulfur dioxygenase that localizes within the mitochondrial matrix. ETHE1 probably plays an important role in metabolic homeostasis in mitochondria. It may also function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. ETHE1 can suppresses p53-induced apoptosis by preventing nuclear localization of RELA. Mutations in ETHE1 gene result in ethylmalonic encephalopathy. Ethylmalonic encephalopathy is an autosomal recessive, invariably fatal disorder characterized by early-onset encephalopathy, microangiopathy, chronic diarrhea, defective cytochrome c oxidase (COX) in muscle and brain, high concentrations of C4 and C5 acylcarnitines in blood and high excretion of ethylmalonic acid in urine.

Overview

A DNA sequence encoding the human ETHE1 (O95571) (Leu13-Ala254) was expressed with a polyhistidine tag at the N-terminus.

Synonyms

HSCO Protein, Human; YF13H12 Protein, Human

Gene Name

Ethylmalonic encephalopathy 1

UniProt

O95571

Expression System

E. coli

Tag

N-His

Endotoxin

Please contact us for more information.

Purity

> 90 % as determined by SDS-PAGE.

Activity

At Leading Biology, the biological activity of a recombinant protein is routinely measured using a bioassay, e.g. chemotaxis or cell proliferation assay, enzyme assay, or a functional ELISA. When a recombinant protein is an enzyme, specific activity is derived from an enzymatic assay. Each enzyme is tested for potency by cleavage of a substrate. We are in the process of updating the biological activity data. If you have any questions regarding this update, please feel free to contact our technical support team.

Form

Lyophilized

Buffer

Lyophilized from sterile PBS, pH 7.4. Please contact us for any concerns or special requirements. Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween 80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.

Molecular Weight

The recombinant human ETHE1 consists of 257 amino acids and predicts a molecular mass of 28.3 KDa. It migrates as an approximately 28 KDa band in SDS-PAGE under reducing conditions.

Storage Conditions

In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid. They are shipped out with blue ice unless customers require otherwise. Stable for 1 year at -20°C or below from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and opening the cap. For long-term storage, aliquot and store at -20°C or below. Avoid repeated freeze-thaw cycles.

Symbol

ETHE1

Species

Human

Uniprot URL

https://www.uniprot.org/uniprot/O95571

AA Sequence

Leu13-Ala254

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