Desmosterol

Desmosterol is a molecule that is part of the sterol family, which are a group of naturally occurring organic compounds. It is a precursor in the biosynthesis of cholesterol, one of the most crucial sterols in the body. Desmosterol is produced in the body through a process known as the Bloch pathway, named after the scientist Konrad Bloch who first described it.

Biosynthesis

Desmosterol is synthesized from lanosterol in a process that involves several enzyme-mediated steps. The first step is the removal of the C-14 methyl group by the enzyme sterol 14-demethylase. This is followed by the isomerization of the double bond from the delta 8(9) position to the delta 7(8) position by the enzyme delta(8)-delta(7) sterol isomerase. The final step is the reduction of the delta 7(8) double bond by the enzyme sterol delta7-reductase, resulting in the formation of desmosterol.

Role in the body

Desmosterol plays a crucial role in the body as a precursor to cholesterol. Cholesterol is a vital component of cell membranes and is also used in the synthesis of steroid hormones, bile acids, and vitamin D. Therefore, the production of desmosterol is a critical step in these important biological processes.

Health implications

Abnormal levels of desmosterol in the body can lead to health problems. For instance, Desmosterolosis, a rare genetic disorder, is characterized by elevated levels of desmosterol. This condition can lead to a variety of symptoms, including developmental delay, growth retardation, and multiple congenital anomalies.

Research

Research into desmosterol has provided valuable insights into the regulation of cholesterol biosynthesis and the potential therapeutic applications of modulating this pathway. For example, some studies suggest that inhibiting the conversion of desmosterol to cholesterol could be a potential strategy for treating atherosclerosis, a disease characterized by the buildup of cholesterol-rich plaques in the arteries.