Recombinant Full Length Human ACO1 Protein, GST-tagged

• Cat.No.: ACO1-782HF
• Product Overview: Human ACO1 full-length ORF ( NP_002188.1, 1 a.a. - 889 a.a.) recombinant protein with GST-tag at N-terminal.

Specification

• Description: The protein encoded by this gene is a bifunctional, cytosolic protein that functions as an essential enzyme in the TCA cycle and interacts with mRNA to control the levels of iron inside cells. When cellular iron levels are high, this protein binds to a 4Fe-4S cluster and functions as an aconitase. Aconitases are iron-sulfur proteins that function to catalyze the conversion of citrate to isocitrate. When cellular iron levels are low, the protein binds to iron-responsive elements (IREs), which are stem-loop structures found in the 5' UTR of ferritin mRNA, and in the 3' UTR of transferrin receptor mRNA. When the protein binds to IRE, it results in repression of translation of ferritin mRNA, and inhibition of degradation of the otherwise rapidly degraded transferrin receptor mRNA. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. Alternative splicing results in multiple transcript variants [provided by RefSeq, Jan 2014]
• Source: In Vitro Cell Free System
• Species: Human
• Tag: GST
• Molecular Mass: 124.8 kDa
• Protein length: 889 amino acids
• AA Sequence: MSNPFAHLAE PLDPVQPGKK FFNLNKLEDS RYGRLPFSIR VLLEAAIRNC DEFLVKKQDI ENILHWNVTQ HKNIEVPFKP ARVILQDFTG VPAVVDFAAM RDAVKKLGGD PEKINPVCPA DLVIDHSIQV DFNRRADSLQ KNQDLEFERN RERFEFLKWG SQAFHNMRII PPGSGIIHQV NLEYLARVVF DQDGYYYPDS LVGTDSHTTM IDGLGILGWG VGGIEAEAVM LGQPISMVLP QVIGYRLMGK PHPLVTSTDI VLTITKHLRQ VGVVGKFVEF FGPGVAQLSI ADRATIANMC PEYGATAAFF PVDEVSITYL VQTGRDEEKL KYIKKYLQAV GMFRDFNDPS QDPDFTQVVE LDLKTVVPCC SGPKRPQDKV AVSDMKKDFE SCLGAKQGFK GFQVAPEHHN DHKTFIYDNT EFTLAHGSVV IAAITSCTNT SNPSVMLGAG LLAKKAVDAG LNVMPYIKTS LSPGSGVVTY YLQESGVMPY LSQLGFDVVG YGCMTCIGNS GPLPEPVVEA ITQGDLVAVG VLSGNRNFEG RVHPNTRANY LASPPLVIAY AIAGTIRIDF EKEPLGVNAK GQQVFLKDIW PTRDEIQAVE RQYVIPGMFK EVYQKIETVN ESWNALATPS DKLFFWNSKS TYIKSPPFFE NLTLDLQPPK SIVDAYVLLN LGDSVTTDHI SPAGNIARNS PAARYLTNRG LTPREFNSYG SRRGNDAVMA RGTFANIRLL NRFLNKQAPQ TIHLPSGEIL DVFDAAERYQ QAGLPLIVLA GKEYGAGSSR DWAAKGPFLL GIKAVLAESY ERIHRSNLVG MGVIPLEYLP GENADALGLT GQERYTIIIP ENLKPQMKVQ VKLDTGKTFQ AVMRFDTDVE LTYFLNGGIL NYMIRKMAK
• Applications: Enzyme-linked Immunoabsorbent Assay
  Western Blot (Recombinant protein)
  Antibody Production
  Protein Array
• Storage: Store at -80 centigrade. Aliquot to avoid repeated freezing and thawing.
• Storage Buffer: 50 mM Tris-HCl, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.

Gene Information

• 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

Q&As

Where is ACO1 found?

ACO1 is found in the mitochondria of eukaryotic cells and in the cytosol of prokaryotic cells.

How is ACO1 assay performed?

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.

What does ACO1 stand for?

How is ACO1 deficiency related to human diseases?

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.

How is ACO1 regulated?

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.

What is the significance of ACO1 in cancer?

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.

What is the function of ACO1?

ACO1 functions as an enzyme that catalyzes the conversion of citrate to isocitrate in the TCA cycle. It also regulates iron homeostasis in cells

What happens when ACO1 is deficient or not functioning properly?

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.