Human Aconitate Hydratase, Mitochondrial (ACO2) Protein
1118€ (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
Aconitate Hydratase, Mitochondrial (ACO2) protein is a recombinant Human protein expressed in Insect.
Documents del producto
Instrucciones
Data sheet
Product specifications
| Category | Proteins and Peptides |
| Immunogen Target | Aconitate Hydratase, Mitochondrial (ACO2) |
| Host | Insect |
| Assay Type | Activity: Not tested Sequence Fragment: Gln28-Gln780 Tag: N-terminal His tag and GST tag |
| Origin | Human |
| Observed MW | 110 kDa |
| Expression | Recombinant |
| Purity | > 85% (SDS-PAGE) |
| Size 1 | 50 µg |
| Form | Lyophilized |
| Tested Applications | SDS-PAGE |
| Buffer | Prior to lyophilization: Sterile 20 mM Tris, 500 mM NaCl, 10% glycerol, pH 8.0, 0.3 mM DTT. |
| Availability | Shipped within 5-15 working days. |
| Storage | Storage: Store lyophilized between -20 °C and -80°C. Stability: Stable when stored reconstituted at 2-8°C for up to 1 week. Reconstituted aliquots are stable at -20°C for up to 3 months. Shelf Life: 12 months. |
| 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 USE IN DIAGNOSTIC, THERAPEUTIC OR COSMETIC PROCEDURES. NOT FOR HUMAN OR ANIMAL CONSUMPTION. Endotoxin Level: < 1.0 EU per µg (LAL method). |
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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