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  Heparin cofactor II

SourceHomo sapiens (human)
Taxonomy Homo sapiens Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo.
Keywords3D-structure; Blood coagulation; Chemotaxis; Complete proteome; Direct protein sequencing; Disease mutation; Glycoprotein; Heparin-binding; Phosphoprotein; Polymorphism; Protease inhibitor; Repeat; Serine protease inhibitor; Signal; Sulfation; Thrombophilia.
Details
Function: Thrombin inhibitor activated by the glycosaminoglycans, heparin or dermatan sulfate. In the presence of the latter, HC-II becomes the predominant thrombin inhibitor in place of antithrombin III (AT-III). Also inhibits chymotrypsin, but in a glycosaminoglycan-independent manner. Peptides at the N-terminal of HC-II have chemotactic activity for both monocytes and neutrophils.

Post-translational modification: Phosphorylation sites are present in the extracelllular medium.

Similarity: Belongs to the serpin family.

Tissue specificity: Expressed predominantly in liver. Also present in plasma.

Disease: Defects in SERPIND1 are the cause of heparin cofactor 2 deficiency (HCF2D)

Sequence length: 499 AA.

Sequence
MKHSLNALLIFLIITSAWGGSKGPLDQLEKGGETAQSADPQWEQLNNKNLSMPLLPADFH
KENTVTNDWIPEGEEDDDYLDLEKIFSEDDDYIDIVDSLSVSPTDSDVSAGNILQLFHGK
SRIQRLNILNAKFAFNLYRVLKDQVNTFDNIFIAPVGISTAMGMISLGLKGETHEQVHSI
LHFKDFVNASSKYEITTIHNLFRKLTHRLFRRNFGYTLRSVNDLYIQKQFPILLDFKTKV
REYYFAEAQIADFSDPAFISKTNNHIMKLTKGLIKDALENIDPATQMMILNCIYFKGSWV
NKFPVEMTHNHNFRLNEREVVKVSMMQTKGNFLAANDQELDCDILQLEYVGGISMLIVVP
HKMSGMKTLEAQLTPRVVERWQKSMTNRTREVLLPKFKLEKNYNLVESLKLMGIRMLFDK
NGNMAGISDQRIAIDLFKHQGTITVNEEGTQATTVTTVGFMPLSTQVRFTVDRPFLFLIY
EHRTSCLLFMGRVANPSRS
Accession NumberP05546 
PubMed ID2894851, 1671335, 15461802, 14702039, 3003690, 3755044, 3907702, 2841345, 1985958, 1939083, 2104620, 16335952, 19159218, 19824718, 2647747, 10391209, 11204559, 15337701 
CEX DBHS_SERPIND1
CTD DB3053
eggNOG DBprNOG06861
Ensembl DBENST00000215727, ENST00000406799
Genecard DBGC22P019452
GeneID DB3053
GermOnline DBENSG00000099937
GO DB0005576, 0008201, 0004867, 0007596, 0006935
HGNC DB4838
HOGENOM DBHBG281494
HPA DBCAB008639
InterPro DBIPR000295, IPR000215
H-InvDBHIX0027857
IPI DBIPI00879573
KEGGhsa:3053
NCBIM12849, AAA52642, M58600, AAA52641, CR456573, CAG30459, AK314200, BAG36878, CH471176.2, EAX02941, X03498, CAA27218, M33660, AAA36185, NP_000176.2
NMPDR fig|9606.3.peg.21291
OMADFHKENT
OMIM105200, 134820, 202400, 134830, 202400, 134850, 202400, 176930, 601367, 134390, 188055, 227400, 600880, 601367, 612309, 227500, 134500, 306700, 300746, 306900, 227600, 176860, 188050, 612283, 612304, 176880, 612336, 264900, 612416, 234000, 610618, 610619, 229000, 612423, 107300, 188050, 134570, 134580, 193400, 277480, 228960, 612358, 176895, 173350, 188050, 217090, 173370, 142360, 188050, 612356
OrthoDBEOG9XPT1N
PDB1JMJ_A, 1JMJ_B, 1JMO_A
PfamPF00079
PharmaGKBPA35053
PROSITE DBPS00284
SMART DBSM00093
UCSCuc002ztb.1
UniGeneHs.474270



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