Species Human Entrez 5046 | Human Mouse Ensembl ENSG00000140479 | |
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Aliases PCSK6, PACE4, SPC4, proprotein convertase subtilisin/kexin type 6 External IDs MGI: 102897 HomoloGene: 20569 GeneCards: PCSK6 | ||
Proprotein convertase subtilisin/kexin type 6 is an enzyme that in humans is encoded by the PCSK6 gene. PCSK6 is a protease that cleaves NODAL into an active form to help trigger the development of left/right (LR) asymmetry. It may also be involved in left and right handedness in humans.
Contents
Function
The protein encoded by this gene belongs to the subtilisin-like proprotein convertase family. The members of this family are proprotein convertases that process latent precursor proteins into their biologically active products. This encoded protein is a calcium-dependent serine endoprotease that can cleave precursor protein at their paired basic amino acid processing sites. Some of its substrates are - transforming growth factor beta related proteins, proalbumin, and von Willebrand factor. Alternatively spliced transcript variants encoding different isoforms have been identified.
PCSK6 also cleaves and activates corin, a serine protease that regulates sodium homeostasis and blood pressure.
Clinical significance
This gene is thought to play a role in tumor progression.
PCSK6 deficiency causes salt-sensitive hypertension in mice.