| Catalog No. | Size | Price (USD) |
| 51015-T60-50 | 50 µL | $147.96 |
| 51015-T60-100 | 100 µL | $251.64 |
| 51015-T60-200 | 200 µL | $355.32 |
General Information
| Product name | Anti-Aconitase 2 Antibody, Rabbit Polyclonal |
| Validated applications | WB (FAQ Protocol), ELISA (FAQ Protocol), IHC-P (FAQ Protocol), IP (FAQ Protocol) |
| Species reactivity | Reacts with: Mouse |
| Specificity | Mouse Aconitase 2 |
| Immunogen | Recombinant Mouse ACO2 / Aconitase 2 protein (Catalog#51015-M20B) |
| Preparation | Produced in rabbits immunized with purified, recombinant Mouse ACO2 / Aconitase 2 (Catalog#51015-M20B; Q99KI0; Gln28-Gln780). ACO2 / Aconitase 2 specific IgG was purified by Mouse ACO2 / Aconitase 2 affinity chromatography. |
| Source | Polyclonal Rabbit IgG |
| Purification | Protein A & Antigen Affinity |
| Formulation | 0.2 μm filtered solution in PBS |
| Conjugate | Unconjugated |
| Form | Liquid |
| Shipping | This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. |
| Storage | This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free. Avoid repeated freeze-thaw cycles. |
Synonyms: Anti-Aco-2 Antibody; Anti-Aco2 Antibody; Anti-Aco3 Antibody; Anti-D10Wsu183e Antibody; Anti-Irp1 Antibody
Image / Experimental Information
| Image 1 Description | Immunochemical staining of mouse ACO2 in mouse skeletal muscle with rabbit polyclonal antibody (1:5000, formalin-fixed paraffin embedded sections). |
| Image 1 Alt Text | Mouse ACO2 / Aconitase 2 Immunohistochemistry(IHC) 3397 |
| Image 1 URL | Source image |
| Image 2 Description | Immunochemical staining of mouse ACO2 in mouse skeletal muscle with rabbit polyclonal antibody (1:5000, formalin-fixed paraffin embedded sections). |
| Image 2 Alt Text | Mouse ACO2 / Aconitase 2 Immunohistochemistry(IHC) 3398 |
| Image 2 URL | Source image |
| Image 3 Description | Immunochemical staining of mouse ACO2 in mouse skeletal muscle with rabbit polyclonal antibody (1:5000, formalin-fixed paraffin embedded sections). |
| Image 3 Alt Text | Mouse ACO2 / Aconitase 2 Immunohistochemistry(IHC) 3399 |
| Image 3 URL | Source image |
| Image 4 Description | Immunochemical staining of mouse ACO2 in mouse skeletal muscle with rabbit polyclonal antibody (1:5000, formalin-fixed paraffin embedded sections). |
| Image 4 Alt Text | Mouse ACO2 / Aconitase 2 Western blot (WB) 13716 |
| Image 4 URL | Source image |
| Image 5 Description | Immunochemical staining of mouse ACO2 in mouse skeletal muscle with rabbit polyclonal antibody (1:5000, formalin-fixed paraffin embedded sections). |
| Image 5 Alt Text | Mouse ACO2 / Aconitase 2 Immunoprecipitation(IP) 15279 |
| Image 5 URL | Source image |
Background Information
| Full Name | aconitase 2, mitochondrial |
| Description | A homozygous missense mutation was identified in the ACO2 gene (c.1240T>G p.Phe414Val) that segregated with HSP complicated by intellectual disability and microcephaly. Lymphoblastoid cell lines of homozygous carrier patients revealed significantly decreased activity of the mitochondrial aconitase enzyme and defective mitochondrial respiration. ACO2 encodes mitochondrial aconitase, an essential enzyme in the Krebs cycle. Recessive mutations in this gene have been previously associated with cerebellar ataxia. We found homozygous or compound heterozygous missense and frameshift mutations in the gene encoding mitochondrial aconitase (ACO2), a tricarboxylic acid cycle enzyme, catalysing interconversion of citrate into isocitrate. Unlike wild type ACO2, all mutant ACO2 proteins failed to complement the respiratory growth of a yeast aco1-deletion strain. The study shows that autosomal recessive ACO2 mutations can cause either isolated or syndromic optic neuropathy. This observation identifies ACO2 as the second gene responsible for non-syndromic autosomal recessive optic neuropathies and provides evidence for a genetic overlap between isolated and syndromic forms, giving further support to the view that optic atrophy is a hallmark of defective mitochondrial energy supply. |
| References |
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