What organ is primarily affected by copper accumulation in Wilson's disease?

Wilson's disease is a genetic disorder characterized by the excessive accumulation of copper in the body, primarily due to a mutation in the ATP7B gene, which is responsible for copper transport. The liver is the primary organ affected by this buildup. In Wilson's disease, copper accumulates in liver cells (hepatocytes), which can lead to hepatocellular damage, liver dysfunction, and ultimately cirrhosis if left untreated.

The liver is crucial for metabolizing copper and secreting it into bile, and when this process is disrupted, copper begins to accumulate within the liver tissues. Over time, this accumulation can spill over into the bloodstream and affect other organs, such as the brain, kidneys, and heart, but the initial and most significant impact occurs in the liver.

In summary, the liver's pivotal role in copper metabolism makes it the primary organ affected by copper accumulation in Wilson's disease.