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Mouse Anti-ACO2 Monoclonal Antibody (V2-5178) (CBMAB-0926-YC)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
V2-5178
Antibody Isotype
IgG2a, κ
Application
ELISA

Basic Information

Immunogen
ACO2 (AAH14092, aa 1-179) partial recombinant protein with GST tag
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
ELISA10 µg/ml

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

Format
Liquid
Buffer
PBS, pH 7.4
Preservative
None
Concentration
Batch dependent
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
aconitase 2, mitochondrial
Introduction
Aconitase 2 is encoded in the nucleus and functions in the mitochondrion. Aconitase 2 belongs to the aconitase/IPM isomerase family which is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle. It was found to be one of the mitochondrial matrix proteins that are preferentially degraded by the serine protease 15(PRSS15), also known as Lon protease, after oxidative modification.
Entrez Gene ID
50
UniProt ID
Q99798
Alternative Names
ICRD; OCA8; OPA9; ACONM; HEL-S-284
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.
More Infomation

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

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