ACO1
Description
The ACO1 gene provides instructions for making aconitase 1, an enzyme crucial for the citric acid cycle, a central metabolic pathway in energy production. This cycle occurs within the mitochondria, often referred to as the "powerhouse" of the cell. Aconitase 1 catalyzes the conversion of citrate to isocitrate, a crucial step in the cycle. Mutations in the ACO1 gene can disrupt this process, leading to various health problems. Understanding the role of ACO1 is essential for comprehending cellular energy metabolism and its implications for human health.
Associated Diseases
Did you know?
Aconitase 1 is a fascinating enzyme that can sense changes in cellular iron levels. When iron levels are low, it can bind to iron-sulfur clusters, which are essential for its activity. This ability to respond to iron levels makes it an important regulator of energy production.
