Reactome | UniProt:P29122 PCSK6

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UniProt:P29122 PCSK6 Show undefined attributes

chain	signal peptide:1-63 propeptide:64-149 chain:150-969
checksum	A3599CC278D09B05
comment	FUNCTION Serine endoprotease that processes various proproteins by cleavage at paired basic amino acids, recognizing the RXXX[KR]R consensus motif. Likely functions in the constitutive secretory pathway, with unique restricted distribution in both neuroendocrine and non-neuroendocrine tissues.COFACTOR The 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. Interacts (immature form including the propeptide) with RCN3; probably involved in the maturation and the secretion of PCSK6 (PubMed:16433634).INTERACTION Not secreted, remains probably in zymogen form in endoplasmic reticulum.SUBCELLULAR LOCATION Not secreted, remains probably in zymogen form in endoplasmic reticulum.SUBCELLULAR LOCATION Retained intracellularly probably through a hydrophobic cluster in their C-terminus.SUBCELLULAR LOCATION Retained intracellularly probably through a hydrophobic cluster in their C-terminus.SUBCELLULAR LOCATION 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.DOMAIN The propeptide domain acts as an intramolecular chaperone assisting the folding of the zymogen within the endoplasmic reticulum. Isoform PACE4D lacks the propeptide domain.MISCELLANEOUS Probably enzymatically inactive.MISCELLANEOUS Probably enzymatically inactive.MISCELLANEOUS Probably enzymatically inactive.MISCELLANEOUS Probably enzymatically inactive.SIMILARITY Belongs to the peptidase S8 family.
created	[InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35
crossReference	[DatabaseIdentifier:11463910] ENSEMBL:ENSG00000140479 [DatabaseIdentifier:12817148] ZINC target:P29122 [DatabaseIdentifier:12813724] ZINC - Substances:PCSK6_HUMAN [DatabaseIdentifier:12810005] ZINC - Predictions - Purchasable:PCSK6_HUMAN [DatabaseIdentifier:11558653] ENSEMBL:ENSP00000478081 [DatabaseIdentifier:11558651] ENSEMBL:ENSP00000482760 [DatabaseIdentifier:11558654] ENSEMBL:ENSP00000477768 [DatabaseIdentifier:11558652] ENSEMBL:ENSP00000479496 [DatabaseIdentifier:11681351] ENSEMBL:ENST00000611716 [DatabaseIdentifier:11681353] ENSEMBL:ENST00000618548 [DatabaseIdentifier:11681350] ENSEMBL:ENST00000611967 [DatabaseIdentifier:11681352] ENSEMBL:ENST00000615296 [DatabaseIdentifier:11780429] Guide to Pharmacology - Targets:2386 [DatabaseIdentifier:11796462] GeneCards:PCSK6 [DatabaseIdentifier:11833653] HMDB Protein:HMDBP01820 [DatabaseIdentifier:11849430] HPA:ENSG00000140479-PCSK6 [DatabaseIdentifier:12134127] OpenTargets:ENSG00000140479 [DatabaseIdentifier:12256660] PRO:P29122 [DatabaseIdentifier:12294292] Pharos - Targets:P29122 [DatabaseIdentifier:12348635] RefSeq:NP_612195.1 [DatabaseIdentifier:12348633] RefSeq:NP_001278238.1 [DatabaseIdentifier:12348636] RefSeq:NP_612192.1 [DatabaseIdentifier:12348638] RefSeq:NP_002561.1 [DatabaseIdentifier:12348637] RefSeq:NP_612198.2 [DatabaseIdentifier:12348639] RefSeq:NP_612197.1 [DatabaseIdentifier:12348634] RefSeq:NP_612196.1
databaseName	UniProt
dbId	403556
description	recommendedName: Proprotein convertase subtilisin/kexin type 6 ecNumber: 3.4.21.- alternativeName: Paired basic amino acid cleaving enzyme 4 alternativeName: Subtilisin-like proprotein convertase 4 shortName: SPC4 alternativeName: Subtilisin/kexin-like protease PACE4
displayName	UniProt:P29122 PCSK6
geneName	PCSK6 PACE4
identifier	P29122
isSequenceChanged	false
keyword	Alternative splicing Calcium Cleavage on pair of basic residues Endoplasmic reticulum Glycoprotein Hydrolase Membrane Protease Proteomics identification Reference proteome Repeat Secreted Serine protease Signal Zymogen
modified	[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
moleculeType	Protein
name	PCSK6
otherIdentifier	11723385_s_at 11734526_a_at 11738236_a_at 11738631_a_at 11742242_at 11748165_a_at 11761162_at 1553531_PM_at 1553531_at 207414_PM_s_at 207414_s_at 210553_PM_x_at 210553_x_at 211262_PM_at 211262_at 211263_PM_s_at 211263_s_at 32001_s_at 3642176 3642177 3642178 3642179 3642180 3642181 3642182 3642190 3642191 3642192 3642193 3642194 3642195 3642197 3642199 3642201 3642203 3642204 3642205 3642206 3642207 3642208 3642209 3642211 3642212 3642213 3642214 3642219 3642220 3642221 3642224 3642230 3642232 3642233 3642234 3642235 3642238 3642239 3642241 3642242 3642243 3642244 3642245 3642246 3642255 3642256 3642257 3642258 3642259 3642260 3642263 3642269 3642270 3642271 3642272 3642281 3642285 3642290 3642300 3642302 3642303 3642304 3642307 3642314 3642315 3642320 3642321 3642322 3642323 3642324 3642326 3642328 5046 7991602 84775_at 90658_s_at 90660_at ADDON002376.hg A_21_P0014797 A_23_P151907 A_23_P390006 A_24_P189997 GE517227 GE59711 GE62904 GO:0004175 GO:0004252 GO:0005515 GO:0005576 GO:0005615 GO:0005783 GO:0005796 GO:0005886 GO:0006508 GO:0007354 GO:0007368 GO:0008201 GO:0008233 GO:0008236 GO:0009100 GO:0009986 GO:0012505 GO:0016020 GO:0016485 GO:0016486 GO:0016787 GO:0030312 GO:0030510 GO:0031012 GO:0032902 GO:0032940 GO:0034369 GO:0048406 GO:1901135 HMNXSV003010216 HMNXSV003030033 HMNXSV003041825 HMNXSV003054015 Hs2.170414.8.S1_3p_at Hs2.170414.8.S1_3p_x_at ILMN_1676384 ILMN_1676595 ILMN_1710070 ILMN_1768577 ILMN_1802550 ILMN_2292592 M80482_at PH_hs_0022325 TC15002009.hg g2330550_3p_a_at g4505576_3p_a_at g666010_3p_at g666012_3p_a_at g666012_3p_x_at p25017 p25946
physicalEntity	[EntityWithAccessionedSequence:R-HSA-1181107] PCSK6 [extracellular region] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-9933752] PCSK6 [Golgi membrane] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-3009047] PCSK6 [Golgi lumen] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-6784630] PCSK6 [extracellular region] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-8849792] PCSK6 [plasma membrane] - Homo sapiens
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:12733335] UCSC:P29122 PCSK6 [ReferenceDNASequence:ensembl:ENSG00000140479] ENSEMBL:ENSG00000140479 PCSK6 [ReferenceDNASequence:11328228] BioGPS Gene:5046 PCSK6 [ReferenceDNASequence:11398842] COSMIC - genes:PCSK6 PCSK6 [ReferenceDNASequence:11425695] CTD Gene:5046 PCSK6 [ReferenceDNASequence:11435725] dbSNP Gene:5046 PCSK6 [ReferenceDNASequence:11828171] HGNC:8569 PCSK6 [ReferenceDNASequence:11870411] KEGG:hsa:5046 PCSK6 [ReferenceDNASequence:11958017] Monarch:5046 PCSK6 [ReferenceDNASequence:12042110] NCBI Gene:5046 PCSK6 [ReferenceDNASequence:12118319] OMIM:167405 PCSK6
referenceTranscript	[ReferenceRNASequence:12530487] RefSeq:NM_138322.4 PCSK6 [ReferenceRNASequence:12530485] RefSeq:NM_138325.3 PCSK6 [ReferenceRNASequence:12530486] RefSeq:NM_138324.3 PCSK6 [ReferenceRNASequence:12530491] RefSeq:NM_001291309.1 PCSK6 [ReferenceRNASequence:12530490] RefSeq:NM_138319.4 PCSK6 [ReferenceRNASequence:12530489] RefSeq:NM_002570.5 PCSK6 [ReferenceRNASequence:12530488] RefSeq:NM_138323.3 PCSK6
schemaClass	ReferenceGeneProduct
secondaryIdentifier	PCSK6_HUMAN Q15099 Q15100 Q9UEG7 Q9UEJ1 Q9UEJ2 Q9UEJ7 Q9UEJ8 Q9UEJ9 Q9Y4G9 Q9Y4H0 Q9Y4H1
sequenceLength	969
species	[Species:48887] Homo sapiens
stId	uniprot:P29122
url	http://purl.uniprot.org/uniprot/P29122

Referrals

(referenceEntity)	[EntityWithAccessionedSequence:R-HSA-1181107] PCSK6 [extracellular region] [EntityWithAccessionedSequence:R-HSA-9933752] PCSK6 [Golgi membrane] [EntityWithAccessionedSequence:R-HSA-3009047] PCSK6 [Golgi lumen] [EntityWithAccessionedSequence:R-HSA-6784630] PCSK6 [extracellular region] [EntityWithAccessionedSequence:R-HSA-8849792] PCSK6 [plasma membrane]
(interactor)	[UndirectedInteraction:11152931] UniProt:P0DTC8 <-> UniProt:P29122 (IntAct)