Cuprimine for Wilson's disease: How does it work? How is it consumed? Evidence? Scientific Studies? Who shouldn't have it? Dosages?
Wilson's disease is an autosomal recessive genetic disorder, which is caused by mutations in a gene called ATP7B. This gene is the gene responsible for regulating the transport of copper and excretion of it outside the body. When this gene has mutations, it leads to copper accumulation inside the body, beginning in places like the liver, and eventually spreading to the brain and kidneys, causing symptoms such as: Hepatitis, cirrhosis, liver failure, tremors, difficulties in speaking, depression, psychosis, personality changes and even copper deposits in the cornea in the shape of rings, and some renal problems. To treat Wilson's disease, cuprimine is administered in patients. It is a chelating agent, meaning that it is a substance which easily bonds with metal ions to form complexes. It bonds to copper ions, turning into a stable complex; this lets it be excreted through urine much more easily. By binding to the ions, cuprimine consumption results in a lower copper concentrati...