ENTRY       EC 2.3.2.13
NAME        Protein-glutamine gamma-glutamyltransferase
            Transglutaminase
            Factor XIIIa
            Fibrinoligase
CLASS       Transferases
            Acyltransferases
            Aminoacyltransferases
SYSNAME     Protein-glutamine:amine gamma-glutamyltransferase
REACTION    Protein glutamine + Alkylamine = Protein N5-alkylglutamine + NH3
SUBSTRATE   Protein glutamine
            Protein lysine
            Glutamine
            Alkylamine
PRODUCT     Protein N5-alkylglutamine
            N5-Alkylglutamine
            NH3
COFACTOR    Calcium
COMMENT     Requires Ca2+. The gamma-carboxamide groups of peptide-bound
            glutamine residues act as acyl donors, and the 6-amino-groups of
            protein- and peptide-bound lysine residues act as acceptors, to
            give intra- and inter-molecular N6-(5-glutamyl)-lysine crosslinks.
            Formed by proteolytic cleavage from plasma Factor XIII.
DISEASE     MIM: 134570  Coagulation factor XIII, A polypeptide; Factor XIIIA
                         deficiency (3)
            MIM: 134580  Coagulation factor XIII, B polypeptide; Factor XIIIB
                         deficiency (3)
            MIM: 190195  Transglutaminase-1 (K polypeptide epidermal type I,
                         protein-glutamine; Ichthyosis, lamellar, autosomal
                         recessive, 242300 (3); Ichthyosiform erythroderma,
                         congenital, 242100 (3)
            MIM: 190196  Transglutaminase-2 (C polypeptide,
            MIM: 230500  Galactosidase, beta-1; GM1-gangliosidosis (3);
                         Mucopolysaccharidosis IVB (3)
            MIM: 600238  Transglutaminase 3 (E polypeptide,
                         protein-glutamin3.2.1.23:
            MIM: 600585  Transglutaminase 4 (prostate)
MOTIF       PS: PS00547  G-Q-[CA]-W-V-x-A-[GA]-V-x(2)-T-x-L-R-C-L-G
GENES       HSA: TGM1(ICR2) TGM2 TGM3(Hs.2022) TGM4(Hs.2387)
STRUCTURES  PDB: 1FIE  1GGT  
DBLINKS     University of Geneva ENZYME DATA BANK: 2.3.2.13
            WIT (What Is There) Metabolic Reconstruction: 2.3.2.13
            SCOP (Structural Classificaion of Proteins): 2.3.2.13
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