Thiosulfate—dithiol sulfurtransferase

Thiosulfate—dithiol sulfurtransferase is an enzyme that plays a crucial role in the metabolism of sulfur-containing compounds within the body. This enzyme catalyzes the transfer of sulfur from thiosulfate to various dithiol compounds, a process that is essential for the biosynthesis of important sulfur-containing biomolecules and for the detoxification of sulfur compounds.

Function

Thiosulfate—dithiol sulfurtransferase operates within the mitochondrial matrix, where it facilitates the transfer of sulfur atoms. This enzymatic action is vital for the synthesis of key sulfur-containing molecules such as biotin and lipoic acid, which are critical for cellular energy production and for the functioning of the citric acid cycle. Additionally, this enzyme plays a significant role in the detoxification pathways for cyanide by converting it to thiocyanate, a less toxic compound that can be excreted from the body.

Structure

The enzyme is composed of multiple subunits, each contributing to its overall function and stability. The precise structure of Thiosulfate—dithiol sulfurtransferase varies among different species, but it generally features a catalytic site where the sulfur transfer reaction occurs. Understanding the structure of this enzyme is crucial for insights into its mechanism of action and for the development of potential therapeutic agents targeting sulfur metabolism disorders.

Clinical Significance

Alterations in the activity of Thiosulfate—dithiol sulfurtransferase can lead to various metabolic disorders, particularly those related to the improper metabolism of sulfur-containing compounds. Deficiencies in this enzyme have been linked to increased susceptibility to toxicity from substances like cyanide, which rely on sulfurtransferase pathways for detoxification. Research into this enzyme also holds potential for understanding certain neurodegenerative diseases and for the development of drugs that can modulate sulfur metabolism for therapeutic purposes.

See Also

• Sulfur metabolism
• Biotin
• Lipoic acid
• Mitochondrial matrix
• Cyanide poisoning