Human Aconitate Hydratase, Mitochondrial (ACO2) Protein
1079€ (50 µg)
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name
Human Aconitate Hydratase, Mitochondrial (ACO2) Protein
category
Proteins and Peptides
provider
Abbexa
reference
abx693569
tested applications
SDS-PAGE
Description
Human ACO2 Protein is a recombinant protein from Human produced in Baculovirus-Insect Cells. A DNA sequence encoding the human ACO2 (Q99798) (Gln 28-Gln 780) was fused with the N-terminal polyhistidine-tagged GST tag at the N-terminus.
Documents del producto
Instrucciones
Data sheet
Product specifications
| Category | Proteins and Peptides |
| Immunogen Target | ACO2 |
| Host | Insect |
| Origin | Human |
| Observed MW | Molecular Weight: 110 kDa Sequence Fragment: Gln28-Gln780 Tag: N-terminal His tag and GST tag Validity: The validity for this protein is 12 months. |
| Expression | Recombinant |
| Purity | > 85% (SDS-PAGE) |
| Size 1 | 50 µg |
| Tested Applications | SDS-PAGE |
| Buffer | Lyophilized from sterile 20mM Tris, 500mM NaCl, 10% glycerol, pH 8.0, 0.3mM DTT. |
| Availability | Shipped within 5-15 working days. |
| Storage | Aliquot and store at -20°C or -80°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q99798 |
| Alias | ICRD,OCA8,OPA9,ACONM,HEL-S-284 |
| Background | Protein ACO2 |
| Status | RUO |
| Note | This product is for research use only. Not for human consumption, cosmetic, therapeutic or diagnostic use. |
Descripción
Aconitase 2 (ACO2) is a mitochondrial enzyme involved in the tricarboxylic acid (TCA) cycle, where it catalyzes the reversible isomerization of citrate to isocitrate via cis-aconitate. ACO2 is essential for cellular energy production, linking carbohydrate metabolism to the production of reducing equivalents such as NADH and FADH2. The enzyme contains an iron-sulfur cluster that is critical for its catalytic activity but also makes it sensitive to oxidative damage, which can impair function. Mutations or dysregulation of ACO2 are associated with mitochondrial diseases, neurodegenerative disorders such as Parkinson's disease, and aging, as oxidative stress often leads to loss of aconitase activity. ACO2 is also being explored as a biomarker for mitochondrial dysfunction in metabolic and neurological conditions.
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ACO2(aconitate hydratase, mitochondrial) is also named as citrate hydro-lyase and belongs to the aconitase/IPM isomerase family. It plays a key function in cellular energy production, and loss of its activity has a major impact on cellular and organismal survival. Western blot shows two bands of 83 kDa and 40 kDa. The 40 kDa fragment decreases with age and oxidative stress, whereas other fragmentation products with molecular weights between 40 and 83 kDa increased with age and MnSOD(mitochondrial manganese superoxide dismutase) deficiency(PMID:12459471). Defects in ACO2 are the cause of infantile cerebellar-retinal degeneration(ICRD).
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