SERPING1
entrez
710
canonical
uniprot
P05155
canonical
ensembl
ENSG00000149131
resolver
ensp
ENSP00000278407
resolver
ensp
ENSP00000549527
resolver
genesymbol
SERPING1
resolver
genesymbol_syn
C1IN
resolver
genesymbol_syn
C1NH
resolver
hgnc
HGNC:1228
resolver
uniprot
A6NMU0
resolver
uniprot
A8KAI9
resolver
uniprot
B2R6L5
resolver
uniprot
B4E1F0
resolver
uniprot
B4E1H2
resolver
uniprot
E9KL26
resolver
uniprot
Q16304
resolver
uniprot
Q547W3
resolver
uniprot
Q59EI5
resolver
uniprot
Q7Z455
resolver
uniprot
Q96FE0
resolver
uniprot
Q9UC49
resolver
uniprot
Q9UCF9
resolver
uniprot_entry
E9KL26_HUMAN
resolver
uniprot_entry
IC1_HUMAN
resolver
uniprot_sec
A6NMU0
resolver
uniprot_sec
A8KAI9
resolver
uniprot_sec
B2R6L5
resolver
uniprot_sec
B4E1F0
resolver
uniprot_sec
B4E1H2
resolver
uniprot_sec
Q16304
resolver
uniprot_sec
Q547W3
resolver
uniprot_sec
Q59EI5
resolver
uniprot_sec
Q7Z455
resolver
uniprot_sec
Q96FE0
resolver
uniprot_sec
Q9UC49
resolver
uniprot_sec
Q9UCF9
resolver
amino_acid_sequence
MASRLTLLTLLLLLLAGDRASSNPNATSSSSQDPESLQDRGEGKVATTVISKMLFVEPILEVSSLPTTNSTTNSATKITANTTDEPTTQPTTEPTTQPTIQPTQPTTQLPTDSPTQPTTGSFCPGPVTLCSDLESHSTEAVLGDALVDFSLKLYHAFSAMKKVETNMAFSPFSIASLLTQVLLGAGENTKTNLESILSYPKDFTCVHQALKGFTTKGVTSVSQIFHSPDLAIRDTFVNASRTLYSSSPRVLSNNSDANLELINTWVAKNTNNKISRLLDSLPSDTRLVLLNAIYLSAKWKTTFDPKKTRMEPFHFKNSVIKVPMMNSKKYPVAHFIDQTLKAKVGQLQLSHNLSLVILVPQNLKHRLEDMEQALSPSVFKAIMEKLEMSKFQPTLLTLPRIKVTTSQDMLSIMEKLEFFDFSYDLNLCGLTEDPDLQVSAMQHQTVLELTETGVEAAAASAISVARTLLVFEVQQPFLFVLWDQQHKFPVFMGRVYDPRA
uniprot
proteins
disease_involvement
DISEASE: Angioedema, hereditary, 1 (HAE1) [MIM:106100]: An autosomal dominant disorder characterized by episodic local swelling involving subcutaneous or submucous tissue of the upper respiratory and gastrointestinal tracts, face, extremities, and genitalia. Hereditary angioedema due to C1 esterase inhibitor deficiency is comprised of two clinically indistinguishable forms. In hereditary angioedema type 1, serum levels of C1 esterase inhibitor are decreased, while in type 2, the levels are normal or elevated, but the protein is non-functional. {ECO:0000269|PubMed:12773530, ECO:0000269|PubMed:1363816, ECO:0000269|PubMed:1451784, ECO:0000269|PubMed:14635117, ECO:0000269|PubMed:16409206, ECO:0000269|PubMed:2118657, ECO:0000269|PubMed:2296585, ECO:0000269|PubMed:22994404, ECO:0000269|PubMed:2365061, ECO:0000269|PubMed:24456027, ECO:0000269|PubMed:3178731, ECO:0000269|PubMed:7814636, ECO:0000269|PubMed:7883978, ECO:0000269|PubMed:8172583, ECO:0000269|PubMed:8529136, ECO:0000269|PubMed:8755917, ECO:0000269|Ref.43}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
function
FUNCTION: 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). {ECO:0000269|PubMed:10946292, ECO:0000269|PubMed:11527969, ECO:0000269|PubMed:3458172, ECO:0000269|PubMed:6416294, ECO:0000269|PubMed:8495195}.
uniprot
proteins
mass_dalton
55154
uniprot
proteins
post_translational_modification
PTM: 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). {ECO:0000269|PubMed:12754519, ECO:0000269|PubMed:14760718, ECO:0000269|PubMed:16040958, ECO:0000269|PubMed:16335952, ECO:0000269|PubMed:17488724, ECO:0000269|PubMed:19139490, ECO:0000269|PubMed:19159218, ECO:0000269|PubMed:19838169, ECO:0000269|PubMed:22171320, ECO:0000269|PubMed:23234360, ECO:0000269|PubMed:3756141}.; PTM: Cleaved by C1S in vitro. {ECO:0000269|PubMed:16169853}.; PTM: (Microbial infection) Can be proteolytically cleaved by E.coli stcE. {ECO:0000269|PubMed:12123444, ECO:0000269|PubMed:15096536}.
uniprot
proteins
protein_family
Serpin family
uniprot
proteins
pubmed
10946292
uniprot
proteins
pubmed
11527969
uniprot
proteins
pubmed
12123444
uniprot
proteins
pubmed
12754519
uniprot
proteins
pubmed
12773530
uniprot
proteins
pubmed
1363816
uniprot
proteins
pubmed
1451784
uniprot
proteins
pubmed
14635117
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
14760718
uniprot
proteins
pubmed
15096536
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16040958
uniprot
proteins
pubmed
16169853
uniprot
proteins
pubmed
16335952
uniprot
proteins
pubmed
16409206
uniprot
proteins
pubmed
16554811
uniprot
proteins
pubmed
17488724
uniprot
proteins
pubmed
19139490
uniprot
proteins
pubmed
19159218
uniprot
proteins
pubmed
19838169
uniprot
proteins
pubmed
2026152
uniprot
proteins
pubmed
2118657
uniprot
proteins
pubmed
2154751
uniprot
proteins
pubmed
22171320
uniprot
proteins
pubmed
2296585
uniprot
proteins
pubmed
22994404
uniprot
proteins
pubmed
23234360
uniprot
proteins
pubmed
2365061
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
24456027
uniprot
proteins
pubmed
3089875
uniprot
proteins
pubmed
3178731
uniprot
proteins
pubmed
3267220
uniprot
proteins
pubmed
3393514
uniprot
proteins
pubmed
3458172
uniprot
proteins
pubmed
3488058
uniprot
proteins
pubmed
3756141
uniprot
proteins
pubmed
6416294
uniprot
proteins
pubmed
7749926
uniprot
proteins
pubmed
7814636
uniprot
proteins
pubmed
7883978
uniprot
proteins
pubmed
8172583
uniprot
proteins
pubmed
8495195
uniprot
proteins
pubmed
8529136
uniprot
proteins
pubmed
8618290
uniprot
proteins
pubmed
8755917
uniprot
proteins
sequence_length
500
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Secreted {ECO:0000269|PubMed:3756141, ECO:0000269|PubMed:6416294}.
uniprot
proteins