Aconitate Hydratase, Mitochondrial (ACO2) Antibody

299€ (50 µl)
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935106861
info@markelab.com
name
Aconitate Hydratase, Mitochondrial (ACO2) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx402121
tested applications
ELISA, IHC
Description
Aconitate Hydratase, Mitochondrial (ACO2) Antibody is a Recombinant Rabbit Monoclonal antibody for the detection of Human ACO2.
Documents del producto
Instrucciones
Data sheet
Product specifications
Category | Primary Antibodies |
Immunogen Target | Aconitate Hydratase, Mitochondrial (ACO2) |
Host | Rabbit |
Reactivity | Human |
Recommended Dilution | IHC: 1/50 - 1/200. Optimal dilutions/concentrations should be determined by the end user. |
Clonality | Monoclonal |
Conjugation | Unconjugated |
Isotype | IgG |
Expression | Recombinant |
Purification | Purified by affinity chromatography. |
Size 1 | 50 µl |
Size 2 | 100 µl |
Form | Liquid |
Tested Applications | ELISA, IHC |
Buffer | PBS, pH 7.4, 150 mM NaCl, 0.02% sodium azide and 50% glycerol. |
Availability | Shipped within 5-10 working days. |
Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
Dry Ice | No |
UniProt ID | Q99798 |
Gene ID | 50 |
Alias | ICRD,OCA8,OPA9,ACONM,HEL-S-284 |
Background | Antibody anti-ACO2 |
Status | RUO |
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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