title: Novel structure elucidation strategy for genetically abnormal fibrinogens with incomplete fibrinopeptide release as applied to fibrinogen Schwarzach.
abstract: A novel and simple strategy was developed for the structure elucidation of those genetically abnormal fibrinogens in which thrombin is unable to release fibrinopeptide A from the abnormal molecules. The method provides evidence for the Arg leads to Cys exchange at the C-terminus of the fibrinopeptide A sequence. The abnormal fibrinogen was mercaptolysed and then S-amino-ethylated. Upon thrombin digestion, the modified fibrinogen released new peptides, as shown by high-performance liquid chromatography. The amino-acid analysis proved that these peptides correspond to the expected fibrinopeptide A variants. It was therefore concluded that the analysed case of dysfibrinogenemia, designated Fibrinogen Schwarzach, contains an A alpha 16 Arg leads to Cys exchange in the heterozygous form.
mesh_terms: D000596:Amino Acids / Q000032:analysis; D001120:Arginine; D002851:Chromatography, High Pressure Liquid; D003545:Cysteine; D005340:Fibrinogen / Q000235:genetics* / Q000378:metabolism*; D015241:Fibrinogens, Abnormal; D005344:Fibrinopeptide A / Q000378:metabolism*; D014644:Genetic Variation; D006579:Heterozygote; D006801:Humans
chemical_list: D000596:Amino Acids; C040461:Fibrinogen Schwarzach; D015241:Fibrinogens, Abnormal; D005344:Fibrinopeptide A; D005340:Fibrinogen; D001120:Arginine; D003545:Cysteine