Selenocysteine insertion sequence-binding protein 2 (SECISBP2)

Selenocysteine insertion sequence-binding protein 2 (SECISBP2) is an essential RNA-binding protein required for the biosynthesis of selenoproteins, which are a family of proteins containing the rare amino acid selenocysteine[1][4][5]. It recognizes and binds to the SECIS element, a specialized RNA structure in the 3′ untranslated region of selenoprotein mRNAs, enabling the recoding of UGA stop codons to specify selenocysteine during translation[1][4]. By forming part of a specialized translational apparatus, SECISBP2 allows the recruitment of the Sec-specific elongation factor (EEFSEC), proper positioning in the ribosome, and protects selenoprotein mRNA from nonsense-mediated decay[6][7]. Mutations in SECISBP2 can cause a multisystem disorder due to the failure of selenoprotein synthesis with manifestations including abnormal thyroid hormone metabolism, myopathy, growth retardation, immunity and antioxidant dysfunction, and, in severe cases, neurological deficits and male infertility[2][3][7]. SECISBP2 is not a classical therapeutic target (such as a receptor or enzyme inhibited or activated by drugs), but understanding its function and deficiency is important for diagnosing and managing affected syndromes and for the broader study of translational regulation and redox biology[1][4][7].

Other names

SECIS binding protein 2SBP2Sec insertion sequence-binding protein 2SECIS-binding protein 2THMA1selenocysteine insertion sequence-binding protein 2