Recombinant Human ACO1, GST-tagged
Cat.No. : | ACO1-9294H |
Product Overview : | Recombinant Human ACO1 protein, fused to GST-tag, was expressed in E.coli and purified by GSH-sepharose. |
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Description : | Aconitase 1, also known as iron regulatory element binding protein 1 (IREB1), is a cytosolic protein which binds to iron-responsive elements (IREs). IREs are stem-loop structures found in the 5 UTR of ferritin mRNA, and in the 3 UTR of transferrin receptor mRNA. The iron-induced binding to the IRE results in repression of translation of ferritin mRNA, and inhibition of degradation of the otherwise rapidly degrading transferrin receptor mRNA. Thus, IREB1 plays a central role in cellular iron homeostasis. It was also shown to have aconitase activity, and hence grouped with the aconitase family of enzymes. |
Source : | E.coli |
Species : | Human |
Tag : | GST |
Protein length : | C-term-350a.a. |
Storage : | The protein is stored in PBS buffer at -20℃. Avoid repeated freezing and thawing cycles. |
Storage Buffer : | 1M PBS (58mM Na2HPO4,17mM NaH2PO4, 68mM NaCl, pH8. ) added with 100mM GSH and 1% Triton X-100,15%glycerol. |
Gene Name : | ACO1 aconitase 1, soluble [ Homo sapiens ] |
Official Symbol : | ACO1 |
Synonyms : | ACO1; aconitase 1, soluble; IREB1; cytoplasmic aconitate hydratase; IREBP; IRP1; IRE-BP 1; aconitate hydratase; citrate hydro-lyase; iron regulatory protein 1; ferritin repressor protein; iron-responsive element binding protein 1; iron-responsive element-binding protein 1; ACONS; IREBP1; |
Gene ID : | 48 |
mRNA Refseq : | NM_002197 |
Protein Refseq : | NP_002188 |
MIM : | 100880 |
UniProt ID : | P21399 |
Chromosome Location : | 9p21.1 |
Pathway : | Citrate cycle (TCA cycle), organism-specific biosystem; Citrate cycle (TCA cycle), conserved biosystem; Citrate cycle, first carbon oxidation, oxaloacetate => 2-oxoglutarate, organism-specific biosystem; Citrate cycle, first carbon oxidation, oxaloacetate => 2-oxoglutarate, conserved biosystem; Glyoxylate and dicarboxylate metabolism, organism-specific biosystem; |
Function : | 4 iron, 4 sulfur cluster binding; 4 iron, 4 sulfur cluster binding; RNA binding; RNA binding; aconitate hydratase activity; aconitate hydratase activity; citrate hydro-lyase (cis-aconitate-forming) activity; iron-responsive element binding; iron-responsiv |
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ACO1-255M | Recombinant Mouse ACO1 Protein, His (Fc)-Avi-tagged | +Inquiry |
ACO1-257H | Recombinant Human ACO1 Protein, His (Fc)-Avi-tagged | +Inquiry |
◆ Lysates | ||
ACO1-510HCL | Recombinant Human ACO1 cell lysate | +Inquiry |
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For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.
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Q&As (8)
Ask a questionACO1 is found in the mitochondria of eukaryotic cells and in the cytosol of prokaryotic cells.
ACO1 assays typically measure the rate of citrate isomerization to isocitrate in the presence of aconitase. This can be done either in vitro using purified ACO1 enzyme, or in a cellular or tissue lysate. The reaction is typically monitored by measuring the absorbance of NADP+ at a wavelength of 340 nm, which decreases as the isocitrate generated in the reaction produces NADPH, a key cofactor in many cellular processes.
Deficiency in ACO1 is a rare genetic disorder that is linked to iron overload and neurodegeneration. This is because ACO1 is involved in the regulation of iron metabolism and the production of antioxidants that protect against oxidative stress. Inherited mutations in the ACO1 gene result in reduced activity of the enzyme, which can alter iron homeostasis and lead to conditions such as hereditary hemochromatosis and Friedreich ataxia.
ACO1 is regulated by a variety of factors, including substrate availability, metal ions such as iron and zinc, post-translational modifications, and interactions with other proteins in cellular signaling pathways.
Recent studies have shown that ACO1 plays a role in cancer progression by regulating cellular metabolism, oxidative stress, and DNA damage repair. ACO1 is overexpressed in several types of cancer, including breast and pancreatic cancer, and has been identified as a potential therapeutic target for cancer treatment. Inhibiting ACO1 can lead to metabolic alterations that impair cancer cell growth and survival.
ACO1 functions as an enzyme that catalyzes the conversion of citrate to isocitrate in the TCA cycle. It also regulates iron homeostasis in cells
When ACO1 is deficient or not functioning properly, it can lead to a variety of health issues. Mutations in the gene that encodes for ACO1 have been linked to hereditary hemochromatosis, a condition characterized by excessive iron absorption and accumulation in organs. ACO1 deficiency has also been associated with oxidative stress, metabolic disorders, and neurodegenerative diseases.
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