Recombinant Human ACO2 Protein, Myc/DDK-tagged, C13 and N15-labeled

• Cat.No.: ACO2-2771H
• Product Overview: ACO2 MS Standard C13 and N15-labeled recombinant protein (NP_001089) with a C-terminal MYC/DDK tag, was expressed in HEK293 cells.

Specification

• Description: The protein encoded by this gene belongs to the aconitase/IPM isomerase family. It is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle. This protein is encoded in the nucleus and functions in the mitochondrion. 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.
• Source: HEK293
• Species: Human
• Tag: Myc/DDK
• Molecular Mass: 85.43 kDa
• AA Sequence: MAPYSLLVTRLQKALGVRQYHVASV LCQRAKVAMSHFEPNEYIHYDLLEK NINIVRKRLNRPLTLSEKIVYGHLD DPASQEIERGKSYLRLRPDRVAMQD ATAQMAMLQFISSGLSKVAVPSTIH CDHLIEAQVGGEKDLRRAKDINQEV YNFLATAGAKYGVGFWKPGSGIIHQ IILENYAYPGVLLIGTDSHTPNGGG LGGICIGVGGADAVDVMAGIPWELK CPKVIGVKLTGSLSGWSSPKDVILK VAGILTVKGGTGAIVEYHGPGVDSI SCTGMATICNMGAEIGATTSVFPYN HRMKKYLSKTGREDIANLADEFKDH LVPDPGCHYDQLIEINLSELKPHIN GPFTPDLAHPVAEVGKVAEKEGWPL DIRVGLIGSCTNSSYEDMGRSAAVA KQALAHGLKCKSQFTITPGSEQIRA TIERDGYAQILRDLGGIVLANACGP CIGQWDRKDIKKGEKNTIVTSYNRN FTGRNDANPETHAFVTSPEIVTALA IAGTLKFNPETDYLTGTDGKKFRLE APDADELPKGEFDPGQDTYQHPPKD SSGQHVDVSPTSQRLQLLEPFDKWD GKDLEDLQILIKVKGKCTTDHISAA GPWLKFRGHLDNISNNLLIGAINIE NGKANSVRNAVTQEFGPVPDTARYY KKHGIRWVVIGDENYGEGSSREHAA LEPRHLGGRAIITKSFARIHETNLK KQGLLPLTFADPADYNKIHPVDKLT IQGLKDFTPGKPLKCIIKHPNGTQE TILLNHTFNETQIEWFRAGSALNRM KELQQTRTRPLEQKLISEEDLAAND ILDYKDDDDKV
• Purity: > 80% as determined by SDS-PAGE and Coomassie blue staining
• Stability: Stable for 3 months from receipt of products under proper storage and handling conditions.
• Storage: Store at -80 centigrade. Avoid repeated freeze-thaw cycles.
• Concentration: 50 μg/mL as determined by BCA
• Storage Buffer: 100 mM glycine, 25 mM Tris-HCl, pH 7.3.

Gene Information

• Gene Name: ACO2 aconitase 2 [ Homo sapiens (human) ]
• Official Symbol: ACO2
• Synonyms: ACO2; aconitase 2, mitochondrial; aconitate hydratase, mitochondrial; ACONM; citrate hydro-lyase; ICRD; MGC20605; MGC33908
• Gene ID: 50
• mRNA Refseq: NM_001098
• Protein Refseq: NP_001089
• MIM: 100850
• UniProt ID: Q99798

Q&As

What is the function of ACO2?

ACO2 is involved in the TCA cycle, which is a critical process for energy production in cells. Specifically, it catalyzes the conversion of citrate to isocitrate, which is a crucial step in the cycle.

Can ACO2 mutations cause disease?

Yes, mutations in the ACO2 gene have been associated with several disorders, including infantile cerebellar-retinal degeneration and mitochondrial encephalopathy. These conditions are characterized by a range of symptoms, including neurological and developmental abnormalities.

Can ACO2 be targeted for medical treatment?

ACO2 is a potential target for cancer treatment, as it is overexpressed in several types of cancer cells. However, the development of drugs that target ACO2 is still in the early stages of research.

How does ACO2 differ from ACO1?

ACO1 and ACO2 are two isoforms of aconitate hydratase, which catalyze the same reaction in the TCA cycle. However, ACO2 is primarily located in the mitochondria of cells, while ACO1 is located in the cytoplasm.

Is ACO2 involved in iron metabolism?

Unlike ACO1, ACO2 is not involved in iron metabolism. Its role is primarily in energy production through the TCA cycle.

What happens if ACO2 is deficient?

ACO2 deficiency has been associated with several disorders, including infantile cerebellar-retinal degeneration and mitochondrial encephalopathy. These conditions are characterized by various neurological and developmental abnormalities.

What is the structure of ACO2?

ACO2 is a homodimeric enzyme composed of two identical subunits. Each subunit contains a catalytic domain and a regulatory domain, which work together to catalyze the isomerization of citrate to isocitrate.

How is ACO2 measured in the laboratory?

ACO2 activity can be measured in the laboratory using a variety of techniques, including spectrophotometric assays and enzymatic activity assays. These assays typically involve measuring the rate of citrate isomerization to isocitrate in the presence of ACO2 and other necessary cofactors.

How is the activity of ACO2 regulated?

ACO2 activity is regulated by a variety of factors, including substrate availability, pH, and post-translational modifications such as phosphorylation. Additionally, ACO2 activity can be modulated by other enzymes and metabolic pathways in the cell.

Does ACO2 have any other functions in addition to energy production?

While ACO2 is primarily known for its role in energy production, recent research suggests that it may also play a role in other cellular processes. For example, studies have shown that ACO2 may be involved in regulating the immune response and cellular metabolism.