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ACO1

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.
Protein class

Cancer-related genes, Citric acid cycle related proteins, Enzymes, Metabolic proteins, Plasma proteins

Predicted location

Intracellular

Single cell type specificity

Cell type enhanced (Hepatocytes, Proximal tubular cells)

Immune cell specificity

Low immune cell specificity

Cell line specificity

Cell line enhanced (ASC diff)

Interaction

Interacts (when associated with the 4Fe-4S) with FBXL5. Interacts with frataxin(81-210).

Molecular function

Lyase, RNA-binding

More Types Infomation

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For Research Use Only. Not For Clinical Use.

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