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Recombinant Human SCO1 protein is a Human Fragment protein, in the 132 to 300 aa range, expressed in Escherichia coli, with >95% purity, < 1 EU/µg endotoxin level and suitable for SDS-PAGE.

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Key facts

Purity
>95% SDS-PAGE
Endotoxin level
< 1 EU/µg
Expression system
Escherichia coli
Tags
GST tag N-Terminus
Applications
SDS-PAGE
Biologically active
No

Amino acid sequence

G S P E F H M G K P L L G G P F S L T T H T G E R K T D K D Y L G Q W L L I Y F G F T H C P D V C P E E L E K M I Q V V D E I D S I T T L P D L T P L F I S I D P E R D T K E A I A N Y V K E F S P K L V G L T G T R E E V D Q V A R A Y R V Y Y S P G P K D E D E D Y I V D H T I I M Y L I G P D G E F L D Y F G Q N K R K G E I A A S I A T H M R P Y R K K S

Reactivity data

Application
SDS-PAGE
Reactivity
Reacts
Dilution info
-
Notes

-

Associated Products

Select an associated product type

1 product for Alternative Product

Target data

Function

Copper metallochaperone essential for the maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Not required for the synthesis of MT-CO2/COX2 but plays a crucial role in stabilizing MT-CO2/COX2 during its subsequent maturation. Involved in transporting copper to the Cu(A) site on MT-CO2/COX2 (PubMed:15229189, PubMed:15659396, PubMed:16735468, PubMed:17189203, PubMed:19336478). Plays an important role in the regulation of copper homeostasis by controlling the abundance and cell membrane localization of copper transporter CTR1 (By similarity).

Alternative names

Recommended products

Recombinant Human SCO1 protein is a Human Fragment protein, in the 132 to 300 aa range, expressed in Escherichia coli, with >95% purity, < 1 EU/µg endotoxin level and suitable for SDS-PAGE.

Key facts

Purity
>95% SDS-PAGE
Endotoxin level
< 1 EU/µg
Expression system
Escherichia coli
Applications
SDS-PAGE
Accession
O75880-1
Animal free
No
Species
Human
Reconstitution
Reconstitute in PBS, pH 7.4
Concentration
Loading...
Storage buffer

pH: 7.2
Constituents: 99% Phosphate Buffer, 0.02% (R*,R*)-1,4-Dimercaptobutan-2,3-diol

Sequence info

Amino acid sequence

G S P E F H M G K P L L G G P F S L T T H T G E R K T D K D Y L G Q W L L I Y F G F T H C P D V C P E E L E K M I Q V V D E I D S I T T L P D L T P L F I S I D P E R D T K E A I A N Y V K E F S P K L V G L T G T R E E V D Q V A R A Y R V Y Y S P G P K D E D E D Y I V D H T I I M Y L I G P D G E F L D Y F G Q N K R K G E I A A S I A T H M R P Y R K K S
Accession
O75880
Protein length
Fragment
Predicted molecular weight
19.2 kDa
Amino acids
132 to 300
Nature
Recombinant
Tags
GST tag N-Terminus

Specifications

Form
Lyophilized
Additional notes

Greater than 95% as determined by reducing SDS-PAGE.

General info

Function

Copper metallochaperone essential for the maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Not required for the synthesis of MT-CO2/COX2 but plays a crucial role in stabilizing MT-CO2/COX2 during its subsequent maturation. Involved in transporting copper to the Cu(A) site on MT-CO2/COX2 (PubMed:15229189, PubMed:15659396, PubMed:16735468, PubMed:17189203, PubMed:19336478). Plays an important role in the regulation of copper homeostasis by controlling the abundance and cell membrane localization of copper transporter CTR1 (By similarity).

Sequence similarities

Belongs to the SCO1/2 family.

Subcellular localisation
Mitochondrion, Mitochondrion inner membrane

Storage

Shipped at conditions
Blue Ice
Appropriate short-term storage duration
A few weeks
Appropriate short-term storage conditions
-20°C
Appropriate long-term storage conditions
-20°C

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.
Activity summary

'SCO1' also known as SCO1 homolog is a protein involved in the assembly of cytochrome c oxidase the last enzyme in the mitochondrial respiratory chain. SCO1 has a mass of approximately 33 kDa and is commonly expressed in tissues with high energy demands such as muscle and brain tissue. The protein plays a mechanical role in copper delivery to cytochrome c oxidase subunits.

Biological function summary

SCO1 participates in the transfer of copper ions essential for the catalytic activity of cytochrome c oxidase. It is a component of the cytochrome c oxidase assembly complex. This function ensures that the enzyme maintains its activity which is necessary for efficient cellular respiration. SCO1 interacts with other mitochondrial proteins responsible for stabilizing complex formation and securing enzyme functionality.

Pathways

SCO1 contributes to the oxidative phosphorylation pathway and is important for the proper functioning of the mitochondrial electron transport chain. Its role in copper ion transfer is vital for energy production in cells. SCO1 interacts with the protein SCO2 another homolog involved in copper binding and transport to cytochrome c oxidase highlighting their interconnected tasks within this pathway.

Associated diseases and disorders

Defects in SCO1 are linked to mitochondrial disorders specifically linked to cytochrome c oxidase deficiency and Leigh syndrome. These conditions involve disruptions in energy metabolism and present with severe clinical manifestations. Mutations in SCO1 can influence its interaction with other proteins such as COX17 essential for copper metabolism exacerbating the mitochondrial dysfunction in affected individuals.

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