TY - JOUR
T1 - Glanzmann thrombasthenia
T2 - A model disease which paved the way to powerful therapeutic agents
AU - Seligsohn, Uri
PY - 2002
Y1 - 2002
N2 - Glanzmann thrombasthenia (GT) is an autosomal recessive bleeding disorder characterized by deficient or dysfunctional glycoprotein (GP) IIb/IIIa compexes. The hallmark of the disease is impaired platelet aggregation stemming from defective fibrinogen binding to GPIIb/IIIa. Based on deciphering the abnormality in GT a monoclonal antibody, peptides and peptidominetic agents, all interfering with fibrinogen binding to GPIIb/III complex, have been developed and successfully used to create a transient thrombasthenia - like state in patients with imminent arterial thrombosis. Currently, the main benefit afforded by these agents has been observed in patients undergoing percutaneous coronary interventions who are at high risk of thrombosis but more indications for their use are evolving.
AB - Glanzmann thrombasthenia (GT) is an autosomal recessive bleeding disorder characterized by deficient or dysfunctional glycoprotein (GP) IIb/IIIa compexes. The hallmark of the disease is impaired platelet aggregation stemming from defective fibrinogen binding to GPIIb/IIIa. Based on deciphering the abnormality in GT a monoclonal antibody, peptides and peptidominetic agents, all interfering with fibrinogen binding to GPIIb/III complex, have been developed and successfully used to create a transient thrombasthenia - like state in patients with imminent arterial thrombosis. Currently, the main benefit afforded by these agents has been observed in patients undergoing percutaneous coronary interventions who are at high risk of thrombosis but more indications for their use are evolving.
KW - Antiplatelet drugs
KW - Glanzmann thrombasthenia
KW - Glycoproteins IIb and IIIa
UR - http://www.scopus.com/inward/record.url?scp=0142072243&partnerID=8YFLogxK
U2 - 10.1159/000073569
DO - 10.1159/000073569
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AN - SCOPUS:0142072243
SN - 1424-8832
VL - 32
SP - 216
EP - 217
JO - Pathophysiology of Haemostasis and Thrombosis
JF - Pathophysiology of Haemostasis and Thrombosis
IS - 5-6
ER -