ACO1
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Official Full Name
aconitase 1, soluble
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Overview
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. -
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-;
- Recombinant Proteins
- Cell & Tissue Lysates
- Protein Pre-coupled Magnetic Beads
- Chicken
- Human
- Mouse
- Rat
- Rhesus Macaque
- Zebrafish
- E.coli
- HEK293
- In Vitro Cell Free System
- Insect Cell
- Mammalian Cell
- Mammalian cells
- Wheat Germ
- Flag
- GST
- His
- His (Fc)
- Avi
- Myc|DDK
- N/A
- N
- Involved Pathway
- Protein Function
- Interacting Protein
- ACO1 Related Articles
ACO1 involved in several pathways and played different roles in them. We selected most pathways ACO1 participated on our site, such as Citrate cycle (TCA cycle), Glyoxylate and dicarboxylate metabolism, Metabolic pathways, which may be useful for your reference. Also, other proteins which involved in the same pathway with ACO1 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
| Pathway Name | Pathway Related Protein |
|---|---|
| Citrate cycle (TCA cycle) | SDHD;SDHC;MDH1;SDHA;IDH3B;FH1;SUCLA2;SDHDA;ACLY |
| Glyoxylate and dicarboxylate metabolism | PCCB;ACAT1;CS;GCSH;CAT;HOGA1;DHDPSL;GLUL;GLULB |
| Metabolic pathways | ST6GALNAC1;FASN;ATP5G3;DLDH;AMY1B;B4GALT2;PIGK;GCH2;POC1B-GALNT4 |
| Carbon metabolism | PDHA1A;TPI1B;HAO2;PDHA2;DLD;FBP1;HKDC1;SDHA;ANI_1_2390104 |
| -Oxocarboxylic acid metabolism | GPT2;ACO2;BCAT2;GOT2B;ACY1;GOT2;IDH3A;GOT1;GPT |
| Biosynthesis of amino acids | TPI1A;THA1;PYCR2;TKTB;PFKMA;ASL2;IDH3A;ALDOCB;ASS1 |
ACO1 has several biochemical functions, for example, 4 iron, 4 sulfur cluster binding, RNA binding, aconitate hydratase activity. Some of the functions are cooperated with other proteins, some of the functions could acted by ACO1 itself. We selected most functions ACO1 had, and list some proteins which have the same functions with ACO1. You can find most of the proteins on our site.
| Function | Related Protein |
|---|---|
| 4 iron, 4 sulfur cluster binding | NDUFS8B;CDKAL1;ACO1;NDUFS2;NTHL1;TYW1B;ACO2;ETFDH;DNA2 |
| RNA binding | CSDC2;PPARGC1B;RPS5;GTF3A;FAU;PAPOLB;TRIM71;PUS3;DDX43 |
| aconitate hydratase activity | ACO1;ACO2 |
| iron-responsive element binding | FECH;ACO1;IREB2 |
| metal ion binding | ZNF609A;FTH1A;GMPR2;KLF13;PRDM1;BMPR1BA;KLF8;SALL3;NR1D4B |
| protein binding | PPP2R5E;ITGA4;CCKBR;CD200R2;DDX19B;DEPDC1;CD244;IL7;GPR143 |
ACO1 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with ACO1 here. Most of them are supplied by our site. Hope this information will be useful for your research of ACO1.
NEB; SGCG; CCDC69; PXN; CLTC; MYBPC1; pbpG; ligB; YBX1
- Q&As
- Reviews
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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