What Is Tiopronin Used For Clinically?

Tiopronin is a well-tolerated medication used to prevent the formation of kidney stones.

It has fewer side effects compared to other drugs such as D-Penicillamine, etc.

It is a prescription thiol drug used primarily in treating severe homozygous cystinuria.

Speaking of cystinuria, it means you have too much cystine in your urine and you are at risk of having stones in your urinary tract.

Tiopronin will help to break the disulfide bond of cystine and binds the sulfhydryl group of the resultant cysteine monomers to form a soluble tiopronin-cysteine-mixed disulfide, which is more water-soluble than cystine and is readily excreted.

This leads to a reduction in urinary cystine concentration and subsequently reduces cystine stone formation.

As a reducing compound with active sulfhydryl groups, it can also chelate heavy metals like NBMI, which has two functional mercapto groups with more potent reducing and chelating abilities.

Animal experiments have shown that tiopronin can prevent liver damage caused by carbon tetrachloride, ethylthioine, and acetaminophen by providing sulfhydryl groups and inhibiting triglyceride accumulation in chronic liver injury.

It can reduce the activity of ATPase in the mitochondria of hepatocytes, thus protecting the hepatic mitochondrial structure and improving liver function.

In addition, it can scavenge free radicals through the reversible binding of sulfhydryl groups to free radicals.