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  Coagulation factor IX

SourceCanis lupus familiaris (dog)
Taxonomy Canis lupus familiaris Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Laurasiatheria; Carnivora; Caniformia; Canidae; Canis.
KeywordsBlood coagulation; Calcium; Cleavage on pair of basic residues; Disease mutation; Disulfide bond; EGF-like domain; Gamma-carboxyglutamic acid; Glycoprotein; Hemophilia; Hydrolase; Hydroxylation; Protease; Repeat; Secreted; Serine protease; Signal; Zymogen.
Details
Function: Factor IX is a vitamin K-dependent plasma protein that participates in the intrinsic pathway of blood coagulation by converting factor X to its active form in the presence of Ca(2 ) ions, phospholipids, and factor VIIIa.

Post-translational modification: Activated by factor XIa, which excises the activation peptide. The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains (By similarity).

Similarity: Belongs to the peptidase S1 family. Contains 2 EGF-like domains. Contains 1 Gla (gamma-carboxy-glutamate) domain. Contains 1 peptidase S1 domain.

Subcellular location: Secreted.

Subunit structure: Heterodimer of a light chain and a heavy chain; disulfide-linked.

Tissue specificity: Synthesized primarily in the liver and secreted in plasma.

Disease: Defects in F9 are the cause of hemophilia B (HEMB).

Sequence length: 452 AA.

Sequence
MAEASGLVTVCLLGYLLSAECAVFLDRENATKILSRPKRYNSGKLEEFVRGNLERECIEE
KCSFEEAREVFENTEKTTEFWKQYVDGDQCESNPCLNDGVCKDDINSYECWCRAGFEGKN
CELDVTCNIKNGRCKQFCKLGPDNKVVCSCTTGYQLAEDQRSCEPAVPFPCGRVSVPHIS
MTRTRAETLFSNMDYENSTEVEKILDNVTQPLNDFTRVVGGKDAKPGQFPWQVLLNGKVD
AFCGGSIINEKWVVTAAHCIEPDVKITIVAGEHNTEKREHTEQKRNVIRTILHHSYNATI
NKYNHDIALLELDEPLTLNSYVTPICIADREYSNIFLKFGSGYVSGWGRVFNKGRSASIL
QYLKVPLVDRATCLRSTKFTIYNNMFCAGFHEGGKDSCQGDSGGPHVTEVEGISFLTGII
SWGEECAMKGKYGIYTKVSRYVNWIKEKTKLT
Accession NumberP19540 
PubMed ID2367529, 2752110, 2481310 
CTD DB404015
eggNOG DBmaNOG10212
Ensembl DBENSCAFT00000030166
GeneID DB404015
GO DB0005576, 0005509, 0004252, 0007596, 0006508
InterPro DBIPR002383, IPR006209, IPR006210, IPR013032, IPR000152, IPR000742, IPR001881, IPR018097, IPR000294, IPR012224, IPR018114, IPR001254, IPR001314, IPR009003
KEGGcfa:404015
NCBIM21757, AAA75006, M33826, AAA30844
OMIM105200, 134820, 202400, 134830, 202400, 134850, 202400, 176930, 601367, 134390, 188055, 227400, 600880, 601367, 612309, 227500, 134500, 306700, 300746, 306900
PfamPF00008, PF00594, PF00089
PROSITE DBPS00010, PS00022, PS01186, PS50026, PS01187, PS00011, PS50998, PS50240, PS00134, PS00135
SMART DBSM00181, SM00179, SM00069, SM00020
UniGeneCfa.3857



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