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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