Fibrinogen Matsumoto V: a variant with Aalpha19 Arg-->Gly (AGG-->GGG). Comparison between fibrin polymerization stimulated by thrombin or reptilase and fibrin monomer polymerization.
Fibrinogen Matsumoto V (M-V) is a dysfibrinogen identified in a 52-year-old woman with systemic lupus erythematous. The triplet AGG encoding the amino acid residue Aalpha19 was replaced by GGG, resulting in the substitution of Arg-->Gly. Residue Aalpha19 has been shown to be one of the most important amino acids in the so-called 'A' site or alpha-chain knob. The thrombin-catalyzed release of fibrinopeptide A from M-V fibrinogen was only slightly delayed yet release of fibrinopeptide B was significantly delayed. Both thrombin-catalyzed fibrin polymerization and fibrin monomer polymerization were markedly impaired compared to normal fibrinogen. In addition, reptilase-catalyzed fibrin polymerization of M-V was much more impaired than thrombin-catalyzed fibrin polymerization. These results indicate 'B' and/or 'b' site of M-V fibrinogen play a more important role in thrombin-catalyzed fibrin polymerization than that of normal control fibrinogen.
Tanaka
H
H
Second Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
Terasawa
F
F
Ito
T
T
Tokunaga
S
S
Ishida
F
F
Kitano
K
K
Kiyosawa
K
K
Okumura
N
N
D012103:Batroxobin / Q000378:metabolism; D001780:Blood Coagulation Tests; D004252:DNA Mutational Analysis; D005260:Female; D005337:Fibrin / Q000378:metabolism; D015241:Fibrinogens, Abnormal / Q000187:drug effects / Q000235:genetics*; D005344:Fibrinopeptide A / Q000378:metabolism; D005345:Fibrinopeptide B / Q000378:metabolism; D014644:Genetic Variation; D006801:Humans; D007700:Kinetics; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000235:genetics; D008875:Middle Aged; D017354:Point Mutation; D013917:Thrombin / Q000378:metabolism
D015241:Fibrinogens, Abnormal; C426983:fibrinogen Matsumoto V; D005344:Fibrinopeptide A; D005345:Fibrinopeptide B; D005337:Fibrin; D012103:Batroxobin; D013917:Thrombin