Description
Key facts
Cell type:U-2 OS,
Species or organism:Human,
Tissue:Bone,
Form:LiquidSee storage information,
Knockout validation:Next Generation Sequencing,Western blot,
Mutation description:Knockout achieved by CRISPR/Cas9 X = 1 bp insertion Frameshift = 100%,
Disease:Osteosarcoma
Product details:
We will provide viable cells that proliferate on revival.
This product is subject to limited use licenses from The Broad Institute, ERS Genomics Limited and Sigma-Aldrich Co. LLC, and is developed with patented technology. For full details of the licenses and patents please refer to our
limited use license
patent pages
Properties and Storage Information:
Gene name-ACO1, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Next Generation Sequencing, Western blot, Shipped at conditions-Dry Ice, Appropriate short-term storage conditions--196°C, Appropriate long-term storage conditions--196°C
Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Aconitase 1 also known as ACO1 or ACOONe is an enzyme with a vital role in cellular metabolism. It weighs approximately 98 kDa and is expressed in the cytosol of cells. Mechanically ACO1 functions as a bifunctional protein participating in the citric acid cycle. Its main role is the reversible isomerization of citrate to isocitrate mediated by the dehydration and rehydration processes. ACO1 requires a 4Fe-4S cluster to be active in its enzymatic form influencing cellular iron metabolism by regulating the levels of iron-responsive elements in mRNA.
Biological function summary
ACO1 connects energy production and iron regulation. It binds to iron-responsive elements when the iron is scarce and acts in its aconitase form when iron levels are sufficient. ACO1 is not part of a larger protein complex but plays an important regulatory role in balancing cellular energy output and iron homeostasis. It affects both glycolysis and the electron transport chain indirectly by altering the availability of citric acid cycle intermediates which are important for ATP production.
Pathways
ACO1 integrates into both the citric acid cycle and the iron regulatory pathway. The citric acid cycle also called the Krebs cycle is fundamental for ATP production and ACO1 serves as a pivotal point within this cycle. Moreover through its iron-regulatory function it connects to iron metabolism pathways that are vital for various cellular processes. ACO1 activity also influences other proteins like ferritin by regulating iron storage and transport mechanisms through its dual functionality.
ACO1 plays a role in certain neurodegenerative diseases and anemia. Dysfunction in ACO1's activity can lead to imbalances in iron metabolism contributing to disorders like Friedreich's ataxia where iron accumulation causes mitochondrial damage. Anemia can result from inadequate iron regulation because of faulty ACO1 function affecting proteins such as transferrin which is important for iron transport in the blood. Understanding ACO1's dual regulatory functions provides essential insights into these disease processes and potential therapeutic approaches.
