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Details on Person UniProt:P05155 SERPING1

Class:IdReferenceGeneProduct:57002
_chainChangeLogsignal peptide:1-22 added on Sat February 7 2015;chain:23-500 added on Sat February 7 2015
_displayNameUniProt:P05155 SERPING1
_timestamp2025-08-15 21:23:22
chainsignal peptide:1-22
chain:23-500
checksum8B5E874833EA6C05
commentFUNCTION Serine protease inhibitor, which acrs as a regulator of the classical complement pathway (PubMed:10946292, PubMed:11527969, PubMed:3458172, PubMed:6416294). Forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases (PubMed:10946292, PubMed:3458172, PubMed:6416294). May also regulate blood coagulation, fibrinolysis and the generation of kinins (PubMed:8495195). Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein (PubMed:8495195).SUBUNIT Interacts with MASP1.SUBUNIT (Microbial infection) Binds to E.coli stcE which allows localization of SERPING1 to cell membranes thus protecting the bacteria against complement-mediated lysis.INTERACTION Highly glycosylated (49%) with N- and O-glycosylation. O-glycosylated with core 1 or possibly core 8 glycans. N-glycan heterogeneity at Asn-25: Hex5HexNAc4 (minor), dHex1Hex5HexNAc4 (minor), Hex6HexNAc5 (major) and dHex1Hex6HexNAc5 (minor).PTM Cleaved by C1S in vitro.PTM (Microbial infection) Can be proteolytically cleaved by E.coli stcE.POLYMORPHISM Chymotrypsin uses Ala-465 as its reactive site in normal plasma protease C1 inhibitor, and His-466 as its reactive site in the variant His-466.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the serpin family.SEQUENCE CAUTION C1-inhibitor entryONLINE INFORMATION SERPING1 mutation db
descriptionrecommendedName: fullName evidence="43"Plasma protease C1 inhibitor shortName: C1 Inh shortName: C1Inh alternativeName: C1 esterase inhibitor alternativeName: fullName evidence="43"C1-inhibiting factor alternativeName: Serpin G1
geneNameSERPING1
C1IN
C1NH
identifierP05155
isSequenceChangedFALSE
keyword3D-structure
Alternative splicing
Blood coagulation
Direct protein sequencing
Disease variant
Disulfide bond
Fibrinolysis
Glycoprotein
Hemostasis
Protease inhibitor
Proteomics identification
Reference proteome
Repeat
Secreted
Serine protease inhibitor
Signal
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameSERPING1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8996639] ENSEMBL:ENSG00000149131 SERPING1 [Homo sapiens]
secondaryIdentifierIC1_HUMAN
A6NMU0
A8KAI9
B2R6L5
B4E1F0
B4E1H2
Q16304
Q547W3
Q59EI5
Q7Z455
Q96FE0
Q9UC49
Q9UCF9
sequenceLength500
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:8970550] UniProt:P05155-1 SERPING1 [Homo sapiens]
[ReferenceIsoform:8970551] UniProt:P05155-2 SERPING1 [Homo sapiens]
[ReferenceIsoform:8970552] UniProt:P05155-3 SERPING1 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:139868] SERPING1 [platelet alpha granule lumen] [Homo sapiens]
[EntityWithAccessionedSequence:158296] SERPING1 [extracellular region] [Homo sapiens]
[EntityWithAccessionedSequence:9650330] SERPING1 P489R [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:9650331] SERPING1 V473M [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:9650346] SERPING1 T189N [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:9650355] SERPING1 Y496* [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:9650362] SERPING1 S170P [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:9650402] SERPING1 A458V [extracellular region] [Homo sapiens]
[EntityWithAccessionedSequence:9650410] SERPING1 Q484* [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:9650415] SERPING1 V288E [endoplasmic reticulum lumen] [Homo sapiens]
List all 46 refering instances
(referenceSequence)[NonsenseMutation:9650325] Nonsense mutation at L-tyrosine 496
[ReplacedResidue:9650327] L-threonine 467 replaced with L-proline
[ReplacedResidue:9650342] L-valine 473 replaced with L-methionine
[ReplacedResidue:9650343] L-serine 170 replaced with L-proline
[ReplacedResidue:9650350] L-arginine 466 replaced with L-leucine
[ReplacedResidue:9650353] L-leucine 177 replaced with L-proline
[FragmentDeletionModification:9650357] Deletion of residues 184 to 228
[NonsenseMutation:9650361] Nonsense mutation at L-glutamine 474
[ReplacedResidue:9650364] L-isoleucine 462 replaced with L-serine
[ReplacedResidue:9650374] L-proline 476 replaced with L-leucine
List all 43 refering instances
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No pathways have been reviewed or authored by UniProt:P05155 SERPING1 (57002)