Anti-SEPSECS antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

The SEPSECS protein also known as SEP (O-phosphoseryl-tRNA:selenocysteinyl-tRNA synthase) plays an important role in selenocysteine biosynthesis. It weighs approximately 53 kDa. This enzyme converts phosphoserine-tRNA^Sec to selenocysteinyl-tRNA^Sec facilitating the incorporation of selenocysteine into proteins. SEPSECS is expressed in various tissues throughout the body including liver kidney and brain making it an important target for understanding protein synthesis involving selenium.

Biological function summary

SEPSECS is involved in the unique process of selenoprotein production. It operates as part of a complex that includes multiple other proteins like SPS1 and EEFSEC which assist in the incorporation of the rare amino acid selenocysteine into selenoproteins. These selenoproteins play important roles in antioxidant defense and redox homeostasis impacting cellular health and stress responses. SEPSECS ensures accuracy in this specialized translation process which is essential for proper cell function.

Pathways

Selenocysteine biosynthesis involves SEPSECS interacting with the SECIS element-binding protein which guides the cellular machinery for incorporating selenocysteine at UGA codons in mRNA that normally signal stop in standard genetic code. This process connects SEPSECS with critical pathways such as selenium metabolism and selenoprotein biosynthesis. The pathway also involves interactions with proteins like selenophosphate synthetase and selenocysteine synthase which work in concert to manage selenium’s incorporation into proteins.

Associated diseases and disorders

SEPSECS mutations can lead to complex neurological conditions including pontocerebellar hypoplasia. These disorders are typically characterized by developmental delays and movement issues linked to improper selenoprotein function. Disruptions in SEPSECS activity can also relate to decreased antioxidant capabilities affecting proteins like glutathione peroxidase and thioredoxin reductase which are important for protecting cells from oxidative stress. Understanding SEPSECS aids in identifying potential therapeutic targets in these conditions.