Antithrombin-III

Details

Name
Antithrombin-III
Kind
protein
Synonyms
  • AT3
  • ATIII
  • Serpin C1
Gene Name
SERPINC1
UniProtKB Entry
P01008Swiss-Prot
Organism
Humans
NCBI Taxonomy ID
9606
Amino acid sequence
>lcl|BSEQ0016052|Antithrombin-III
MYSNVIGTVTSGKRKVYLLSLLLIGFWDCVTCHGSPVDICTAKPRDIPMNPMCIYRSPEK
KATEDEGSEQKIPEATNRRVWELSKANSRFATTFYQHLADSKNDNDNIFLSPLSISTAFA
MTKLGACNDTLQQLMEVFKFDTISEKTSDQIHFFFAKLNCRLYRKANKSSKLVSANRLFG
DKSLTFNETYQDISELVYGAKLQPLDFKENAEQSRAAINKWVSNKTEGRITDVIPSEAIN
ELTVLVLVNTIYFKGLWKSKFSPENTRKELFYKADGESCSASMMYQEGKFRYRRVAEGTQ
VLELPFKGDDITMVLILPKPEKSLAKVEKELTPEVLQEWLDELEEMMLVVHMPRFRIEDG
FSLKEQLQDMGLVDLFSPEKSKLPGIVAEGRDDLYVSDAFHKAFLEVNEEGSEAAASTAV
VIAGRSLNPNRVTFKANRPFLVFIREVPLNTIIFMGRVANPCVK
Number of residues
464
Molecular Weight
52601.935
Theoretical pI
6.68
GO Classification
Functions
identical protein binding
Processes
regulation of blood coagulation
Components
collagen-containing extracellular matrix / endoplasmic reticulum lumen
General Function
Most important serine protease inhibitor in plasma that regulates the blood coagulation cascade (PubMed:15140129, PubMed:15853774). AT-III inhibits thrombin, matriptase-3/TMPRSS7, as well as factors IXa, Xa and XIa (PubMed:15140129). Its inhibitory activity is greatly enhanced in the presence of heparin
Specific Function
heparin binding
Pfam Domain Function
Signal Regions
1-32
Transmembrane Regions
Not Available
Cellular Location
Secreted, extracellular space
Gene sequence
>lcl|BSEQ0016053|Antithrombin-III (SERPINC1)
ATGTATTCCAATGTGATAGGAACTGTAACCTCTGGAAAAAGGAAGGTTTATCTTTTGTCC
TTGCTGCTCATTGGCTTCTGGGACTGCGTGACCTGTCACGGGAGCCCTGTGGACATCTGC
ACAGCCAAGCCGCGGGACATTCCCATGAATCCCATGTGCATTTACCGCTCCCCGGAGAAG
AAGGCAACTGAGGATGAGGGCTCAGAACAGAAGATCCCGGAGGCCACCAACCGGCGTGTC
TGGGAACTGTCCAAGGCCAATTCCCGCTTTGCTACCACTTTCTATCAGCACCTGGCAGAT
TCCAAGAATGACAATGATAACATTTTCCTGTCACCCCTGAGTATCTCCACGGCTTTTGCT
ATGACCAAGCTGGGTGCCTGTAATGACACCCTCCAGCAACTGATGGAGGTATTTAAGTTT
GACACCATATCTGAGAAAACATCTGATCAGATCCACTTCTTCTTTGCCAAACTGAACTGC
CGACTCTATCGAAAAGCCAACAAATCCTCCAAGTTAGTATCAGCCAATCGCCTTTTTGGA
GACAAATCCCTTACCTTCAATGAGACCTACCAGGACATCAGTGAGTTGGTATATGGAGCC
AAGCTCCAGCCCCTGGACTTCAAGGAAAATGCAGAGCAATCCAGAGCGGCCATCAACAAA
TGGGTGTCCAATAAGACCGAAGGCCGAATCACCGATGTCATTCCCTCGGAAGCCATCAAT
GAGCTCACTGTTCTGGTGCTGGTTAACACCATTTACTTCAAGGGCCTGTGGAAGTCAAAG
TTCAGCCCTGAGAACACAAGGAAGGAACTGTTCTACAAGGCTGATGGAGAGTCGTGTTCA
GCATCTATGATGTACCAGGAAGGCAAGTTCCGTTATCGGCGCGTGGCTGAAGGCACCCAG
GTGCTTGAGTTGCCCTTCAAAGGTGATGACATCACCATGGTCCTCATCTTGCCCAAGCCT
GAGAAGAGCCTGGCCAAGGTAGAGAAGGAACTCACCCCAGAGGTGCTGCAAGAGTGGCTG
GATGAATTGGAGGAGATGATGCTGGTGGTCCACATGCCCCGCTTCCGCATTGAGGACGGC
TTCAGTTTGAAGGAGCAGCTGCAAGACATGGGCCTTGTCGATCTGTTCAGCCCTGAAAAG
TCCAAACTCCCAGGTATTGTTGCAGAAGGCCGAGATGACCTCTATGTCTCAGATGCATTC
CATAAGGCATTTCTTGAGGTAAATGAAGAAGGCAGTGAAGCAGCTGCAAGTACCGCTGTT
GTGATTGCTGGCCGTTCGCTAAACCCCAACAGGGTGACTTTCAAGGCCAACAGGCCTTTC
CTGGTTTTTATAAGAGAAGTTCCTCTGAACACTATTATCTTCATGGGCAGAGTAGCCAAC
CCTTGTGTTAAGTAA
Chromosome Location
1
Locus
1q25.1
External Identifiers
ResourceLink
UniProtKB IDP01008
UniProtKB Entry NameANT3_HUMAN
GenBank Protein ID179161
GenBank Gene IDM21642
GeneCard IDSERPINC1
GenAtlas IDSERPINC1
HGNC IDHGNC:775
PDB ID(s)1ANT, 1ATH, 1AZX, 1BR8, 1DZG, 1DZH, 1E03, 1E04, 1E05, 1JVQ, 1LK6, 1NQ9, 1OYH, 1R1L, 1SR5, 1T1F, 1TB6, 2ANT, 2B4X, 2B5T, 2BEH, 2GD4, 2HIJ, 2ZNH, 3EVJ, 3KCG, 4EB1
KEGG IDhsa:462
IUPHAR/Guide To Pharmacology ID2632
NCBI Gene ID462
General References
  1. Bock SC, Wion KL, Vehar GA, Lawn RM: Cloning and expression of the cDNA for human antithrombin III. Nucleic Acids Res. 1982 Dec 20;10(24):8113-25. [Article]
  2. Chandra T, Stackhouse R, Kidd VJ, Woo SL: Isolation and sequence characterization of a cDNA clone of human antithrombin III. Proc Natl Acad Sci U S A. 1983 Apr;80(7):1845-8. [Article]
  3. Olds RJ, Lane DA, Chowdhury V, De Stefano V, Leone G, Thein SL: Complete nucleotide sequence of the antithrombin gene: evidence for homologous recombination causing thrombophilia. Biochemistry. 1993 Apr 27;32(16):4216-24. [Article]
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  31. Chang JY, Tran TH: Antithrombin III Basel. Identification of a Pro-Leu substitution in a hereditary abnormal antithrombin with impaired heparin cofactor activity. J Biol Chem. 1986 Jan 25;261(3):1174-6. [Article]
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  38. Gandrille S, Aiach M, Lane DA, Vidaud D, Molho-Sabatier P, Caso R, de Moerloose P, Fiessinger JN, Clauser E: Important role of arginine 129 in heparin-binding site of antithrombin III. Identification of a novel mutation arginine 129 to glutamine. J Biol Chem. 1990 Nov 5;265(31):18997-9001. [Article]
  39. Austin RC, Rachubinski RA, Blajchman MA: Site-directed mutagenesis of alanine-382 of human antithrombin III. FEBS Lett. 1991 Mar 25;280(2):254-8. [Article]
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  60. Picard V, Dautzenberg MD, Villoutreix BO, Orliaguet G, Alhenc-Gelas M, Aiach M: Antithrombin Phe229Leu: a new homozygous variant leading to spontaneous antithrombin polymerization in vivo associated with severe childhood thrombosis. Blood. 2003 Aug 1;102(3):919-25. Epub 2003 Feb 20. [Article]
  61. Nagaizumi K, Inaba H, Amano K, Suzuki M, Arai M, Fukutake K: Five novel and four recurrent point mutations in the antithrombin gene causing venous thrombosis. Int J Hematol. 2003 Jul;78(1):79-83. [Article]
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  63. Kuhli C, Jochmans K, Scharrer I, Luchtenberg M, Hattenbach LO: Retinal vein occlusion associated with antithrombin deficiency secondary to a novel G9840C missense mutation. Arch Ophthalmol. 2006 Aug;124(8):1165-9. [Article]
  64. Puurunen M, Salo P, Engelbarth S, Javela K, Perola M: Type II antithrombin deficiency caused by a founder mutation Pro73Leu in the Finnish population: clinical picture. J Thromb Haemost. 2013 Oct;11(10):1844-9. doi: 10.1111/jth.12364. [Article]

Associated Data

Drug Relations
DrugDrug groupPharmacological action?TypeActionsDetails
Fondaparinuxapproved, investigationalyestargetpotentiatorDetails
Heparinapproved, investigationalyestargetpotentiatorDetails
EnoxaparinapprovedyestargetpotentiatorDetails
SR-123781AinvestigationalunknowntargetDetails
Sulodexideapproved, investigationalyestargetpotentiatorDetails
Ardeparinapproved, investigational, withdrawnyestargetpotentiatorDetails
N-FormylmethionineexperimentalyestargetinhibitorDetails
TinzaparinapprovedyestargetpotentiatorDetails
Nadroparinapproved, investigationalyestargetpotentiatorDetails
DalteparinapprovedyestargetpotentiatorDetails
Antithrombin III humanapprovedunknowntargetagonistDetails
Copperapproved, investigationalunknowntargetDetails
Bemiparinapproved, investigationalunknowntargetantagonistDetails
Danaparoidapproved, withdrawnyestargetpositive allosteric modulatorDetails
Protamine sulfateapprovedunknowntargetDetails
DelparantaginvestigationalyestargetantagonistDetails