DLST Human
Description
Product Specs
Product Science Overview
Dihydrolipoamide S-Succinyltransferase, also known as DLST, is a crucial enzyme in the human body. It is a component of the 2-oxoglutarate dehydrogenase complex, which plays a significant role in the citric acid cycle (also known as the Krebs cycle or TCA cycle). This enzyme is essential for cellular respiration, a process that generates energy in the form of adenosine triphosphate (ATP) by oxidizing acetyl-CoA derived from carbohydrates, fats, and proteins.
Dihydrolipoamide S-Succinyltransferase is a mitochondrial protein that belongs to the 2-oxoacid dehydrogenase family. It is one of the three core components of the 2-oxoglutarate dehydrogenase complex, along with the E1 (2-oxoglutarate dehydrogenase) and E3 (dihydrolipoamide dehydrogenase) components . The primary function of DLST is to catalyze the transfer of a succinyl group from succinyl-CoA to dihydrolipoamide, forming succinyl-dihydrolipoamide and releasing CoA-SH .
The DLST gene is located on chromosome 14 in humans and encodes the dihydrolipoamide S-succinyltransferase protein . This gene is highly conserved across different species, indicating its essential role in cellular metabolism. Alternative splicing of the DLST gene results in multiple transcript variants, which may lead to different isoforms of the protein .
Mutations in the DLST gene have been associated with various metabolic disorders. For instance, defects in the DLST gene can lead to Pheochromocytoma/Paraganglioma Syndrome 7 and Hereditary Paraganglioma-Pheochromocytoma Syndromes . These conditions are characterized by the development of tumors in the adrenal glands and other parts of the body. Additionally, the enzyme’s role in the citric acid cycle makes it a potential target for therapeutic interventions in metabolic diseases and cancer .
Recombinant DLST refers to the enzyme produced through recombinant DNA technology, which involves inserting the DLST gene into a suitable expression system, such as bacteria or yeast, to produce the protein in large quantities. This recombinant protein is used in various research applications, including studying the enzyme’s structure and function, investigating its role in metabolic pathways, and developing potential therapeutic agents.
