NP_001278238.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
105,375 Da
NCBI Official Full Name
proprotein convertase subtilisin/kexin type 6 isoform i preproprotein
NCBI Official Synonym Full Names
proprotein convertase subtilisin/kexin type 6
NCBI Official Synonym Symbols
NCBI Protein Information
proprotein convertase subtilisin/kexin type 6; paired basic amino acid cleaving enzyme 4; paired basic amino acid cleaving system 4; subtilisin-like proprotein convertase 4; subtilisin/kexin-like protease PACE4
UniProt Protein Name
Proprotein convertase subtilisin/kexin type 6
UniProt Synonym Protein Names
Paired basic amino acid cleaving enzyme 4; Subtilisin-like proprotein convertase 4; SPC4; Subtilisin/kexin-like protease PACE4
UniProt Synonym Gene Names
UniProt Entry Name
PCSK6_HUMAN
NCBI Summary for PACE4
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. [provided by RefSeq, Feb 2014]
UniProt Comments for PACE4
PCSK6: 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. Belongs to the peptidase S8 family. 8 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted, signal peptide; Secreted; EC 3.4.21.-; Protease
Chromosomal Location of Human Ortholog: 15q26.3
Cellular Component: extracellular matrix; extracellular space; cell surface; membrane; Golgi lumen; endoplasmic reticulum
Molecular Function: heparin binding; serine-type endopeptidase activity; endopeptidase activity; nerve growth factor binding
Biological Process: nerve growth factor processing; glycoprotein metabolic process; nerve growth factor receptor signaling pathway; zygotic determination of anterior/posterior axis, embryo; regulation of BMP signaling pathway; peptide hormone processing; protein processing; proteolysis; determination of left/right symmetry; nerve growth factor production; secretion by cell
Research Articles on PACE4
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Pathways associated with anti-PACE4 antibody
Diseases associated with anti-PACE4 antibody
Organs/Tissues associated with anti-PACE4 antibody
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