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MGP002191

UniProt Annotations

Entry Information
Gene Nameproprotein convertase subtilisin/kexin type 6
Protein EntryPCSK6_HUMAN
UniProt IDP29122
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=8; Name=PACE4A-I; Synonyms=PACE4; IsoId=P29122-1; Sequence=Displayed; Name=PACE4A-II; IsoId=P29122-2; Sequence=VSP_005436; Name=PACE4B; Synonyms=PACE4.1; IsoId=P29122-3; Sequence=VSP_005428, VSP_005429; Note=Probably enzymatically inactive.; Name=PACE4C; IsoId=P29122-4; Sequence=VSP_005432, VSP_005433; Note=Probably enzymatically inactive.; Name=PACE4CS; IsoId=P29122-5; Sequence=VSP_005430, VSP_005431; Note=Probably enzymatically inactive.; Name=PACE4D; IsoId=P29122-6; Sequence=VSP_005427, VSP_005434, VSP_005435; Note=Probably enzymatically inactive.; Name=PACE4E-I; IsoId=P29122-7; Sequence=VSP_005437; Name=PACE4E-II; IsoId=P29122-8; Sequence=VSP_005436, VSP_005437;
Catalytic ActivityRelease of mature proteins from their proproteins by cleavage of Arg-Xaa-Yaa-Arg-|-Zaa bonds, where Xaa can be any amino acid and Yaa is Arg or Lys.
CofactorName=Ca(2+); Xref=ChEBI:CHEBI:29108; Evidence={ECO:0000305};
DomainThe propeptide domain acts as an intramolecular chaperone assisting the folding of the zymogen within the endoplasmic reticulum. Isoform PACE4D lacks the propeptide domain.
FunctionLikely 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.
SimilarityBelongs to the peptidase S8 family. {ECO:0000305}.
SimilarityContains 1 peptidase S8 domain. {ECO:0000305}.
SimilarityContains 1 PLAC domain. {ECO:0000255|PROSITE- ProRule:PRU00233}.
SimilarityContains 5 FU (furin-like) repeats. {ECO:0000305}.
Subcellular LocationIsoform PACE4A-II: Secreted.
Subcellular LocationIsoform PACE4A-I: Secreted.
Subcellular LocationIsoform PACE4B: Secreted.
Subcellular LocationIsoform PACE4C: Endoplasmic reticulum. Note=Not secreted, remains probably in zymogen form in endoplasmic reticulum.
Subcellular LocationIsoform PACE4CS: Endoplasmic reticulum. Note=Not secreted, remains probably in zymogen form in endoplasmic reticulum.
Subcellular LocationIsoform PACE4E-I: Endomembrane system; Peripheral membrane protein. Note=Retained intracellularly probably through a hydrophobic cluster in their C-terminus.
Subcellular LocationIsoform PACE4E-II: Endomembrane system; Peripheral membrane protein. Note=Retained intracellularly probably through a hydrophobic cluster in their C-terminus.
SubunitThe PACE4A-I precursor protein seems to exist in the reticulum endoplasmic as both a monomer and a dimer-sized complex whereas mature PACE4A-I exists only as a monomer, suggesting that propeptide cleavage affects its tertiary or quaternary structure.
Tissue SpecificityEach 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.
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