PROS1
entrez
5627
canonical
uniprot
P07225
canonical
ensembl
ENSG00000184500
resolver
ensp
ENSP00000330021
resolver
ensp
ENSP00000377783
resolver
ensp
ENSP00000497376
resolver
enst
ENST00000348974
resolver
enst
ENST00000394236
resolver
enst
ENST00000650591
resolver
genesymbol
PROS1
resolver
genesymbol_syn
PROS
resolver
hgnc
HGNC:9456
resolver
uniprot
A0A0S2Z4K3
resolver
uniprot
A0A0S2Z4L3
resolver
uniprot
A0A3B3ISQ0
resolver
uniprot
A8KAC9
resolver
uniprot
D3DN28
resolver
uniprot
Q15518
resolver
uniprot
Q7Z715
resolver
uniprot
Q9UCZ8
resolver
uniprot_entry
A0A0S2Z4K3_HUMAN
resolver
uniprot_entry
A0A0S2Z4L3_HUMAN
resolver
uniprot_entry
A0A3B3ISQ0_HUMAN
resolver
uniprot_entry
PROS_HUMAN
resolver
uniprot_sec
A8KAC9
resolver
uniprot_sec
D3DN28
resolver
uniprot_sec
Q15518
resolver
uniprot_sec
Q7Z715
resolver
uniprot_sec
Q9UCZ8
resolver
amino_acid_sequence
MRVLGGRCGALLACLLLVLPVSEANFLSKQQASQVLVRKRRANSLLEETKQGNLERECIEELCNKEEAREVFENDPETDYFYPKYLVCLRSFQTGLFTAARQSTNAYPDLRSCVNAIPDQCSPLPCNEDGYMSCKDGKASFTCTCKPGWQGEKCEFDINECKDPSNINGGCSQICDNTPGSYHCSCKNGFVMLSNKKDCKDVDECSLKPSICGTAVCKNIPGDFECECPEGYRYNLKSKSCEDIDECSENMCAQLCVNYPGGYTCYCDGKKGFKLAQDQKSCEVVSVCLPLNLDTKYELLYLAEQFAGVVLYLKFRLPEISRFSAEFDFRTYDSEGVILYAESIDHSAWLLIALRGGKIEVQLKNEHTSKITTGGDVINNGLWNMVSVEELEHSISIKIAKEAVMDINKPGPLFKPENGLLETKVYFAGFPRKVESELIKPINPRLDGCIRSWNLMKQGASGIKEIIQEKQNKHCLVTVEKGSYYPGSGIAQFHIDYNNVSSAEGWHVNVTLNIRPSTGTGVMLALVSGNNTVPFAVSLVDSTSEKSQDILLSVENTVIYRIQALSLCSDQQSHLEFRVNRNNLELSTPLKIETISHEDLQRQLAVLDKAMKAKVATYLGGLPDVPFSATPVNAFYNGCMEVNINGVQLDLDEAISKHNDIRAHSCPSVWKKTKNS
uniprot
proteins
disease_involvement
DISEASE: Thrombophilia due to protein S deficiency, autosomal dominant (THPH5) [MIM:612336]: A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. Based on the plasma levels of total and free PROS1 as well as the serine protease-activated protein C cofactor activity, three types of THPH5 have been described: type I, characterized by reduced total and free PROS1 levels together with reduced anticoagulant activity; type III, in which only free PROS1 antigen and PROS1 activity levels are reduced; and the rare type II which is characterized by normal concentrations of both total and free PROS1 antigen, but low cofactor activity. {ECO:0000269|PubMed:10447256, ECO:0000269|PubMed:10613647, ECO:0000269|PubMed:10706858, ECO:0000269|PubMed:10790208, ECO:0000269|PubMed:11372770, ECO:0000269|PubMed:11776305, ECO:0000269|PubMed:11858485, ECO:0000269|PubMed:11927129, ECO:0000269|PubMed:12351389, ECO:0000269|PubMed:12632031, ECO:0000269|PubMed:15238143, ECO:0000269|PubMed:15712227, ECO:0000269|PubMed:18322254, ECO:0000269|PubMed:7482398, ECO:0000269|PubMed:7545463, ECO:0000269|PubMed:7579449, ECO:0000269|PubMed:7803790, ECO:0000269|PubMed:8298131, ECO:0000269|PubMed:8639833, ECO:0000269|PubMed:8701404, ECO:0000269|PubMed:8765219, ECO:0000269|PubMed:8781426, ECO:0000269|PubMed:8943854, ECO:0000269|PubMed:8977443, ECO:0000269|PubMed:9031443, ECO:0000269|PubMed:9241758, ECO:0000269|Ref.15}. Note=The disease is caused by variants affecting the gene represented in this entry.; DISEASE: Thrombophilia due to protein S deficiency, autosomal recessive (THPH6) [MIM:614514]: A very rare and severe hematologic disorder resulting in thrombosis and secondary hemorrhage usually beginning in early infancy. Some affected individuals develop neonatal purpura fulminans, multifocal thrombosis, or intracranial hemorrhage. {ECO:0000269|PubMed:20484936}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
function
FUNCTION: Anticoagulant plasma protein; it is a cofactor to activated protein C in the degradation of coagulation factors Va and VIIIa. It helps to prevent coagulation and stimulating fibrinolysis.
uniprot
proteins
mass_dalton
75123
uniprot
proteins
mutagenesis
MUTAGEN 515; /note="R->A,E: Markedly reduced secretion of the mutant."; /evidence="ECO:0000269|PubMed:8639833"; MUTAGEN 515; /note="R->K: No change in secretion of the mutant."; /evidence="ECO:0000269|PubMed:8639833
uniprot
proteins
post_translational_modification
PTM: The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains. {ECO:0000250}.
uniprot
proteins
pubmed
10447256
uniprot
proteins
pubmed
10613647
uniprot
proteins
pubmed
10706858
uniprot
proteins
pubmed
10790208
uniprot
proteins
pubmed
11372770
uniprot
proteins
pubmed
11776305
uniprot
proteins
pubmed
11858485
uniprot
proteins
pubmed
11927129
uniprot
proteins
pubmed
12351389
uniprot
proteins
pubmed
12632031
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15238143
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15712227
uniprot
proteins
pubmed
15952784
uniprot
proteins
pubmed
16335952
uniprot
proteins
pubmed
16959974
uniprot
proteins
pubmed
18322254
uniprot
proteins
pubmed
20484936
uniprot
proteins
pubmed
2143091
uniprot
proteins
pubmed
2148110
uniprot
proteins
pubmed
2148111
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
27535533
uniprot
proteins
pubmed
2820795
uniprot
proteins
pubmed
2944113
uniprot
proteins
pubmed
3467362
uniprot
proteins
pubmed
7482398
uniprot
proteins
pubmed
7545463
uniprot
proteins
pubmed
7579449
uniprot
proteins
pubmed
7803790
uniprot
proteins
pubmed
8298131
uniprot
proteins
pubmed
8639833
uniprot
proteins
pubmed
8701404
uniprot
proteins
pubmed
8765219
uniprot
proteins
pubmed
8781426
uniprot
proteins
pubmed
8943854
uniprot
proteins
pubmed
8977443
uniprot
proteins
pubmed
9031443
uniprot
proteins
pubmed
9241758
uniprot
proteins
sequence_length
676
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Secreted.
uniprot
proteins