prohormone convertase
Also written as: PC — prohormone convertase
An enzyme that cleaves prohormones into their active peptide forms within neuroendocrine cells.
Full Definition
Prohormone convertases (PCs) are a family of serine endoproteases responsible for the post-translational processing of prohormones and proneuropeptides into their biologically active forms. The two major subtypes, PC1/3 and PC2, have distinct cleavage specificities and tissue distributions, with PC1/3 primarily found in endocrine cells and PC2 in neural tissues. These enzymes cleave at basic amino acid residues (lysine and arginine) within the regulated secretory pathway, followed by carboxypeptidase E action to remove C-terminal basic residues. Deficiencies in prohormone convertases can lead to severe neuroendocrine disorders, including early-onset obesity, diabetes, and adrenal insufficiency, highlighting their critical role in hormone maturation.
Usage
Usage note: Specify subtype (PC1/3, PC2, etc.) when precision is required; also called proprotein convertase in some contexts.
In Context
- "Prohormone convertase 1/3 deficiency was suspected given the elevated proinsulin-to-insulin ratio." — Endocrine genetics consultation
- "Immunostaining revealed reduced prohormone convertase 2 expression in the hypothalamic neurons." — Neuropathology report