[Detection of platelet fibronectin from congenital fibrinogenopenic patients and its clinical significance].
OBJECTIVE
To assess the platelet and plasma concentrations of fibronectin (Fn) and fibrinogen (Fg) in congenital fibrinogenopenic (FgP) patients and explore their role in inducing platelet adhesion and aggregation.


METHODS
A FgP family was selected as study group and the platelets isolated and purified to assess concentrations of Fn and Fg in platelets, alpha-granules and plasma with Western blotting, immuofluoresence staining and flow cytometry (FACS), respectively, the expression of platelets GP II b/III a by FACS.


RESULTS
The concentration of platelets Fn in FgP patients is higher than that in controls, and is higher in homozygote than in heterozygote. In contrast, plasma Fn levels were identical in all samples. The amount of platelet Fg from FgP patients is lower than that from the controls and positively correlated with the concentration of their plasma Fg. No difference in the expression of platelet GP II b/III a had been found.


CONCLUSIONS
It suggested that increased platelet Fn could partially compensate the lack of Fg and lead the platelet adhesion and aggregation.
Ding
Kaiyang
K
Department of Hematology, Anhui provincial Hospital, Hefei 230001, China.
Wu
Jingsheng
J
Zhai
Zhimin
Z
Xu
Xiucai
X
Sun
Zimin
Z
Wang
Mingli
M
Ni
Heyu
H
D000347:Afibrinogenemia / Q000151:congenital / Q000378:metabolism* / Q000473:pathology; D001792:Blood Platelets / Q000378:metabolism* / Q000473:pathology; D002448:Cell Adhesion / Q000502:physiology; D005260:Female; D005340:Fibrinogen / Q000235:genetics / Q000378:metabolism*; D005353:Fibronectins / Q000097:blood / Q000235:genetics / Q000378:metabolism*; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D008297:Male; D010375:Pedigree; D010974:Platelet Aggregation / Q000502:physiology; D010980:Platelet Membrane Glycoproteins / Q000378:metabolism
D005353:Fibronectins; D010980:Platelet Membrane Glycoproteins; D005340:Fibrinogen