Anti-Aconitase 2 antibody [6D1BE4]

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Aconitase 2 also known as ACO2 is an enzyme involved in the citric acid cycle which plays an important role in energy production within the cell. This enzyme converts citrate to isocitrate via a cis-aconitate intermediate an essential step in the cycle that generates ATP. Aconitase 2 has a molecular mass of approximately 83 kDa and is primarily expressed in the mitochondria of eukaryotic cells where it functions optimally due to the organelle's environment.

Biological function summary

Aconitase 2 supports cellular energy metabolism by facilitating the conversion of citrate derivatives. It is an iron-sulfur protein integral to both the cytosolic aconitase and mitochondrial varieties that functions as part of the mitochondrial aconitase complex. The enzyme's iron-sulfur cluster gets oxidized under different conditions causing the enzyme to act as a regulatory factor in response to iron levels further linking it with cellular iron metabolism.

Pathways

Aconitase 2 plays a significant role in the tricarboxylic acid (TCA) cycle commonly known as the Krebs cycle and the iron regulatory pathway. In the TCA cycle aconitase 2 acts after citrate synthase and before isocitrate dehydrogenase establishing its role in the middle of the cycle. It is functionally related to iron regulatory proteins particularly IRP1 which can interchangeably bind to iron-responsive elements or possess aconitase activity in its Fe-containing form.

Associated diseases and disorders

Disruptions in aconitase 2 activity are linked to conditions like Friedreich's ataxia and cancer. Friedreich's ataxia involves mitochondrial dysfunction wherein the decreased activity of aconitase 2 leads to impaired energy metabolism contributing to neurodegeneration. In cancers altered aconitase 2 function can impact cellular proliferation by modifying energy metabolism pathways. Aconitase 2 also interacts with the protein frataxin in the context of these conditions underlining its involvement in disease mechanisms related to mitochondrial dysfunction and iron metabolism.