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Recombinant Human Superoxide Dismutase 4/CCS protein is a Human Full Length protein, in the 1 to 274 aa range, expressed in Escherichia coli, with >90% purity and suitable for SDS-PAGE, MS.

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Images

SDS-PAGE - Recombinant Human Superoxide Dismutase 4/CCS protein (AB99300), expandable thumbnail

Key facts

Purity
>90% SDS-PAGE
Expression system
Escherichia coli
Tags
His tag N-Terminus
Applications
SDS-PAGE, MS
Biologically active
No

Amino acid sequence

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

Reactivity data

Application
SDS-PAGE
Reactivity
Reacts
Dilution info
-
Notes

-

Application
MS
Reactivity
Reacts
Dilution info
-
Notes

-

Target data

Function

Delivers copper to copper zinc superoxide dismutase (SOD1).

Alternative names

Recommended products

Recombinant Human Superoxide Dismutase 4/CCS protein is a Human Full Length protein, in the 1 to 274 aa range, expressed in Escherichia coli, with >90% purity and suitable for SDS-PAGE, MS.

Key facts

Purity
>90% SDS-PAGE
Expression system
Escherichia coli
Applications
SDS-PAGE, MS
Accession
O14618-1
Animal free
No
Species
Human
Concentration
Loading...
Storage buffer

pH: 8
Constituents: 10% Glycerol (glycerin, glycerine), 1.16% Sodium chloride, 0.316% Tris HCl, 0.0154% (R*,R*)-1,4-Dimercaptobutan-2,3-diol

Sequence info

Amino acid sequence

M G S S H H H H H H S S G L V P R G S H M A S D S G N Q G T L C T L E F A V Q M T C Q S C V D A V R K S L Q G V A G V Q D V E V H L E D Q M V L V H T T L P S Q E V Q A L L E G T G R Q A V L K G M G S G Q L Q N L G A A V A I L G G P G T V Q G V V R F L Q L T P E R C L I E G T I D G L E P G L H G L H V H Q Y G D L T N N C N S C G N H F N P D G A S H G G P Q D S D R H R G D L G N V R A D A D G R A I F R M E D E Q L K V W D V I G R S L I I D E G E D D L G R G G H P L S K I T G N S G E R L A C G I I A R S A G L F Q N P K Q I C S C D G L T I W E E R G R P I A G K G R K E S A Q P P A H L
Accession
O14618
Protein length
Full Length
Predicted molecular weight
31.2 kDa
Amino acids
1 to 274
Nature
Recombinant
Tags
His tag N-Terminus

Specifications

Form
Liquid
Additional notes

ab99300 is purified using conventional chromatography techniques.

General info

Function

Delivers copper to copper zinc superoxide dismutase (SOD1).

Sequence similarities

In the C-terminal section; belongs to the Cu-Zn superoxide dismutase family.

Post-translational modifications

Ubiquitinion by XIAP/BIRC4 leads to enhancement of its chaperone activity toward its physiologic target, SOD1, rather than proteasomal degradation. XIAP/BIRC4 preferentially ubiquitinates at Lys-241.

Storage

Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
-20°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle

Notes

This product was previously labelled as Superoxide Dismutase 4.

Supplementary info

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

Superoxide Dismutase 4 also known as CCS or Copper Chaperone for SOD1 plays a mechanical role as a copper chaperone for the antioxidant enzyme SOD1 (Superoxide Dismutase 1). With a molecular mass of approximately 30 kDa CCS assists in inserting copper into SOD1 activating it for its enzymatic function. CCS is expressed in various tissues including the brain liver and muscle facilitating its role wherever SOD1 activity is needed for protection against oxidative damage.

Biological function summary

CCS contributes significantly to oxidative stress response by catalyzing the dismutation of superoxide radicals into less reactive molecular oxygen and hydrogen peroxide. This protein does not function independently but is part of a complex system involving SOD1 to mitigate oxidative damage within cells. CCS ensures SOD1 acquires the necessary copper which is essential for its dismutase activity hence playing a critical role in maintaining cellular health.

Pathways

The copper delivery to SOD1 by CCS integrates into the broader antioxidant defense pathway. The copper chaperones like CCS link to pathways involving cellular copper homeostasis and oxidative stress management. The protein SOD1 which relies on CCS for copper addition also aligns with the apoptosis and neuroprotective pathways highlighting a shared role in cellular oxidation states control and maintaining neuronal integrity.

Associated diseases and disorders

CCS associates with conditions such as Amyotrophic Lateral Sclerosis (ALS) and certain neurodegenerative disorders. These conditions relate to the malfunction of the antioxidant defense often involving misfolded SOD1 due to improper copper loading. The impairment in CCS’s role affects not just SOD1 but potentially other copper-dependent proteins exacerbating stress conditions and cellular vulnerability that characterizes these diseases.

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1 product image

  • SDS-PAGE - Recombinant Human Superoxide Dismutase 4/CCS protein (ab99300), expandable thumbnail

    SDS-PAGE - Recombinant Human Superoxide Dismutase 4/CCS protein (ab99300)

    15% SDS-PAGE showing ab299300 (3μg).

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Product protocols

For this product, it's our understanding that no specific protocols are required. You can:

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