Question:medium
Bernard-Soulier syndrome is due to a deficiency of which platelet glycoprotein?
Bernard-Soulier syndrome is due to a deficiency of which platelet glycoprotein?
Show Hint
Think of the platelet receptor that grabs von Willebrand factor for adhesion.
Updated On: Jun 24, 2026
- Gp IIb/IIIa
- Gp Ib
- von Willebrand factor (vWF)
- TNF
Show Solution
The Correct Option is B
Solution and Explanation
Step 1: Recall that primary haemostasis needs platelets to stick to exposed collagen, a process bridged by von Willebrand factor. Step 2: The platelet anchor that grips vWF is the glycoprotein Ib-IX-V receptor. Step 3: In Bernard-Soulier syndrome this $Gp\,Ib$ receptor is missing or non-functional, so platelets cannot latch onto vWF and adhesion collapses, giving large platelets with low counts. Step 4: Contrast this with Glanzmann thrombasthenia, where $Gp\,IIb/IIIa$ (the fibrinogen-binding aggregation receptor) is absent; that is a different disease. A primary vWF shortage is von Willebrand disease, and TNF is an inflammatory cytokine, so neither fits. Therefore the deficient protein here is Gp Ib.\[\boxed{\text{Gp Ib}}\]
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