Mouse Recombinant ACO2 protein, GST Tag (V2LY-0526-LY7769)

Name: Mouse Recombinant ACO2 protein, GST Tag
SKU: V2LY-0526-LY7769
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Expressed Host

Baculovirus-Insect Cells

Protein Species

Mouse

Tag

GST Tag

Protein Construction

This product is Mouse Recombinant ACO2 protein, GST Tag consist of Amino Acid: 28-780 and predicts a molecular mass of 110 kDa.

Molecule Mass

110 kDa

Sequence

Amino Acid: 28-780

Species

Mouse

Formulations & Storage [For reference only, actual COA shall prevail!]

Purity

>90% as determined by SDS-PAGE

Endotoxin

Please contact us for more information.

Format

Lyophilized

Reconstitution

Allow the vial and reconstitution buffer to equilibrate to room temperature. Briefly centrifuge or tap down the vial to ensure that all lyophilized powder is collected at the bottom of the vial. For the reconstitution of this product, we recommend adding PBS or sterile water to achieve a final antibody concentration of 1 mg/mL. Allow the vial to reconstitute for 10-15 minutes at room temperature with gentle agitation. Avoid vigorous shaking that can cause foaming and antibody denaturation. Aliquot into volumes based on your experiment and store liquid protein at -20°C or -80°C for long time.

Buffer

Lyophilized from sterile PBS, Trehalose, Arginine, Chaps

Preservative

None

Storage

Samples are stable for up to twelve months from date of receipt at -20°C to -80°C. Store it under sterile conditions at -20°C to -80°C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.

More Infomation

Target

Full Name

aconitase 2, mitochondrial

Function

Catalyzes the isomerization of citrate to isocitrate via cis-aconitate.

Biological Process

Citrate metabolic process
Generation of precursor metabolites and energy
Tricarboxylic acid cycle

Cellular Location

Mitochondrion

Involvement in disease

Infantile cerebellar-retinal degeneration (ICRD): A severe autosomal recessive neurodegenerative disorder characterized by onset between ages 2 and 6 months of truncal hypotonia, athetosis, seizures, and ophthalmologic abnormalities, particularly optic atrophy and retinal degeneration. Affected individuals show profound psychomotor retardation, with only some achieving rolling, sitting, or recognition of family. Brain MRI shows progressive cerebral and cerebellar degeneration.
Optic atrophy 9 (OPA9): A condition that features progressive visual loss in association with optic atrophy. Atrophy of the optic disk indicates a deficiency in the number of nerve fibers which arise in the retina and converge to form the optic disk, optic nerve, optic chiasm and optic tracts.

PTM

Forms covalent cross-links mediated by transglutaminase TGM2, between a glutamine and the epsilon-amino group of a lysine residue, forming homopolymers and heteropolymers.

Palmieri, E. M., Gonzalez-Cotto, M., Baseler, W. A., Davies, L. C., Ghesquière, B., Maio, N., ... & McVicar, D. W. (2020). Nitric oxide orchestrates metabolic rewiring in M1 macrophages by targeting aconitase 2 and pyruvate dehydrogenase. Nature communications, 11(1), 1-17.

Ciccarone, F., Di Leo, L., Lazzarino, G., Maulucci, G., Di Giacinto, F., Tavazzi, B., & Ciriolo, M. R. (2020). Aconitase 2 inhibits the proliferation of MCF-7 cells promoting mitochondrial oxidative metabolism and ROS/FoxO1-mediated autophagic response. British journal of cancer, 122(2), 182-193.

Ciccarone, F., De Falco, P., & Ciriolo, M. R. (2020). Aconitase 2 sensitizes MCF-7 cells to cisplatin eliciting p53-mediated apoptosis in a ROS-dependent manner. Biochemical Pharmacology, 180, 114202.

Fukada, M., Yamada, K., Eda, S., Inoue, K., Ohba, C., Matsumoto, N., ... & Nakayama, A. (2019). Identification of novel compound heterozygous mutations in ACO2 in a patient with progressive cerebral and cerebellar atrophy. Molecular genetics & genomic medicine, 7(7), e00698.

Marelli, C., Hamel, C., Quiles, M., Carlander, B., Larrieu, L., Delettre, C., ... & Guissart, C. (2018). ACO2 mutations: A novel phenotype associating severe optic atrophy and spastic paraplegia. Neurology Genetics, 4(2).

Kelman, J. C., Kamien, B. A., Murray, N. C., Goel, H., Fraser, C. L., & Grigg, J. R. (2018). A sibling study of isolated optic neuropathy associated with novel variants in the ACO2 gene. Ophthalmic genetics, 39(5), 648-651.

Bouwkamp, C. G., Afawi, Z., Fattal-Valevski, A., Krabbendam, I. E., Rivetti, S., Masalha, R., ... & Kushner, S. A. (2018). ACO2 homozygous missense mutation associated with complicated hereditary spastic paraplegia. Neurology Genetics, 4(2).

Abela, L., Spiegel, R., Crowther, L. M., Klein, A., Steindl, K., Papuc, S. M., ... & Simmons, T. L. (2017). Plasma metabolomics reveals a diagnostic metabolic fingerprint for mitochondrial aconitase (ACO2) deficiency. PloS one, 12(5), e0176363.

Srivastava, S., Gubbels, C. S., Dies, K., Fulton, A., Yu, T., & Sahin, M. (2017). Increased survival and partly preserved cognition in a patient with ACO2-related disease secondary to a novel variant. Journal of child neurology, 32(9), 840-845.

Chen, C. M., Wu, Y. R., & Chang, K. H. (2017). Altered aconitase 2 activity in Huntington’s disease peripheral blood cells and mouse model striatum. International journal of molecular sciences, 18(11), 2480.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Alternative Versions

Human Recombinant ACO2 protein, GST Tag (CAT#: V2LY-0526-LY1901)

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Isotype control

For research use only. Not intended for any clinical use.

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