Structure and properties of clots from fibrinogen Bicêtre II (gamma 308 Asn-->Lys). Increased permeability due to larger pores, thicker fibers, and decreased rigidity.
Fibrinogen Bicêtre II is a dysfibrinogenemia in which there is a substitution of Lys for Asn at gamma 308. We have studied the polymerization of this abnormal fibrinogen by measurement of turbidity and have characterized clot structure by scanning electron microscopy, permeation, and viscoelastic measurements. The results of these studies demonstrate that this amino acid substitution has substantial effects on the structure and properties of the clot, resulting in clots made up of thick fibers and large pores with greatly reduced stiffness and increased slippage of protofibrils.
Marchi
R
R
Laboratorio de Fisiopatología, Centro de Medicina Experimental, IVIC, Caracas, Venezuela.
Loyau
S
S
Anglés-Cano
E
E
Weisel
J W
JW
D015241:Fibrinogens, Abnormal / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D009154:Mutation
D015241:Fibrinogens, Abnormal