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  Coagulation factor XIII A chain

SourceHomo sapiens (human)
Taxonomy Homo sapiens Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo.
Keywords3D-structure; Acetylation; Acyltransferase; Blood coagulation; Calcium; Complete proteome; Cytoplasm; Direct protein sequencing; Disease mutation; Glycoprotein; Metal-binding; Polymorphism; Secreted; Transferase; Zymogen.
Details
Function: Factor XIII is activated by thrombin and calcium ion to a transglutaminase that catalyzes the formation of gamma-glutamyl-epsilon-lysine cross-links between fibrin chains, thus stabilizing the fibrin clot. Also cross-link alpha-2-plasmin inhibitor, or fibronectin, to the alpha chains of fibrin.

Post-translational modification: The activation peptide is released by thrombin.

Similarity: Belongs to the transglutaminase superfamily. Transglutaminase family.

Subcellular location: Cytoplasm. Secreted. Note=Secreted into the blood plasma. Cytoplasmic in most tissues, but also secreted in the blood plasma.

Subunit structure: Tetramer of two A chains and two B chains.

Disease: Defects in F13A1 are the cause of F13A deficiency

Sequence length: 732 AA.

Sequence
MSETSRTAFGGRRAVPPNNSNAAEDDLPTVELQGVVPRGVNLQEFLNVTSVHLFKERWDT
NKVDHHTDKYENNKLIVRRGQSFYVQIDFSRPYDPRRDLFRVEYVIGRYPQENKGTYIPV
PIVSELQSGKWGAKIVMREDRSVRLSIQSSPKCIVGKFRMYVAVWTPYGVLRTSRNPETD
TYILFNPWCEDDAVYLDNEKEREEYVLNDIGVIFYGEVNDIKTRSWSYGQFEDGILDTCL
YVMDRAQMDLSGRGNPIKVSRVGSAMVNAKDDEGVLVGSWDNIYAYGVPPSAWTGSVDIL
LEYRSSENPVRYGQCWVFAGVFNTFLRCLGIPARIVTNYFSAHDNDANLQMDIFLEEDGN
VNSKLTKDSVWNYHCWNEAWMTRPDLPVGFGGWQAVDSTPQENSDGMYRCGPASVQAIKH
GHVCFQFDAPFVFAEVNSDLIYITAKKDGTHVVENVDATHIGKLIVTKQIGGDGMMDITD
TYKFQEGQEEERLALETALMYGAKKPLNTEGVMKSRSNVDMDFEVENAVLGKDFKLSITF
RNNSHNRYTITAYLSANITFYTGVPKAEFKKETFDVTLEPLSFKKEAVLIQAGEYMGQLL
EQASLHFFVTARINETRDVLAKQKSTVLTIPEIIIKVRGTQVVGSDMTVTVQFTNPLKET
LRNVWVHLDGPGVTRPMKKMFREIRPNSTVQWEEVCRPWVSGHRKLIASMSSDSLRHVYG
ELDVQIQRRPSM
Accession NumberP00488 
PubMed ID3026437, 2877457, 2901091, 14574404, 15489334, 2877456, 4811064, 16335952, 7913750, 7660355, 9515726, 9988734, 7913909, 1353995, 9763561, 10391209 
CEX DBHS_F13A1
CTD DB2162
DIP DBDIP-377N
eggNOG DBprNOG18947
Ensembl DBENST00000264870
CATHG3DSA:2.60.40.30, G3DSA:2.60.40.10
Genecard DBGC06M006089
GeneID DB2162
GermOnline DBENSG00000124491
GO DB0005576, 0031093, 0008415, 0005509, 0003810, 0007596
HGNC DB3531
HOGENOM DBHBG444754
HPA DBCAB002155, HPA001804
InterPro DBIPR008957, IPR013783, IPR014756, IPR002931, IPR008958, IPR013808, IPR001102
IPI DBIPI00297550
KEGGhsa:2162
NCBIM14539, AAA52489, M14354, AAA52488, M22001, AAA52415, M21987, M21988, M21989, M21990, M21991, M21992, M21993, M21995, M21996, M21997, M21998, M21999, M22000, AB208852, BAD92089, AF418272, AAL12161, AL157775.15, CAC36886.3, AL133326.10, AL391420.16, CAI39797.2, CAO03607, BC027963, AAH27963, NP_000120.2
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
Orphanet DB331
PDB1EVU_A, 1EVU_B, 1EX0_A, 1EX0_B, 1F13_A, 1F13_B, 1FIE_A, 1FIE_B, 1GGT_A, 1GGT_B, 1GGU_A, 1GGU_B, 1GGY_A, 1GGY_B, 1QRK_A, 1QRK_B
PfamPF00927, PF01841, PF00868
PharmaGKBPA162
PROSITE DBPS00547
SMART DBSM00460
UniGeneHs.335513



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