Anti-PACE4/PCSK6 Antibody Picoband® Fluoro488 Conjugated

Proprotein convertase subtilisin/kexin type 6 is an enzyme that in humans is encoded by the PCSK6 gene. This gene encodes a member of the subtilisin-like proprotein convertase family, which includes proteases that process protein and peptide precursors trafficking through regulated or constitutive branches of the secretory pathway. The encoded protein undergoes an initial autocatalytic processing event in the ER to generate a heterodimer which exits the ER and sorts to the trans-Golgi network where a second autocatalytic event takes place and the catalytic activity is acquired. The encoded protease is constitutively secreted into the extracellular matrix and expressed in many tissues, including neuroendocrine, liver, gut, and brain. This gene encodes one of the seven basic amino acid-specific members which cleave their substrates at single or paired basic residues. Some of its substrates include transforming growth factor beta related proteins, proalbumin, and von Willebrand factor. This gene is thought to play a role in tumor progression and left-right patterning. Alternatively spliced transcript variants encoding different isoforms have been identified.

Proprotein convertase subtilisin/kexin type 6;3.4.21.-; Paired basic amino acid cleaving enzyme 4; Subtilisin-like proprotein convertase 4; SPC4; Subtilisin/kexin-like protease PACE4; PCSK6; PACE4

Each PACE4 isoform exhibits a unique restricted distribution. Isoform PACE4A-I is expressed in heart, brain, placenta, lung, skeletal muscle, kidney, pancreas, but at comparatively higher levels in the liver. Isoform PACE4A-II is at least expressed in placenta. Isoform PACE4B was only found in the embryonic kidney cell line from which it was isolated. Isoform PACE4C and isoform PACE4D are expressed in placenta. Isoform PACE4E-I is expressed in cerebellum, placenta and pituitary. Isoform PACE4E-II is at least present in cerebellum.

Likely to represent an endoprotease activity within the constitutive secretory pathway, with unique restricted distribution in both neuroendocrine and non-neuroendocrine tissues and capable of cleavage at the RX (K/R) R consensus motif.

1. Entrez Gene: PCSK6 proprotein convertase subtilisin/kexin type 6. 2. Constam DB, Robertson EJ (May 1, 2000) . SPC4/PACE4 regulates a TGFbeta signaling network during axis formation.. Genes & Development 14 (9) : 1146–55. 3. Scerri TS, Brandler WM, Paracchini S, Morris AP, Ring SM, Richardson AJ, Talcott JB, Stein J, Monaco AP (Feb 1, 2011) . PCSK6 is associated with handedness in individuals with dyslexia.. Human Molecular Genetics 20 (3) : 608–14.