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Alan Nurden: Inherited GPIV (CD36) Deficiency Linked to Bleeding for the First Time
Alan Nurden/curegt.org
Sep 6, 2026, 12:27

Alan Nurden: Inherited GPIV (CD36) Deficiency Linked to Bleeding for the First Time

Alan Nurden, Emeritus Research Director at CNRS, Co-Founder of the French National Reference Centre for Inherited Platelet Disorders (CRPP), shared a post on LinkedIn about a recent article by Loredana Bury et al, published in Biomolecules, adding:

“As someone who has lived through the whole period of investigations on platelet membrane glycoproteins, beginning with the ISTH congress in Oslo in 1971 when I presented as a very nervous student in the first oral communication session on the subject along with such pioneers as Aaron Marcus and Graham Jamieson, I found much pleasure in reading this classic paper by Loredana Bury and her colleagues on inherited defects of GPIV (CD36) in patients with bleeding of unknown cause.

This paper combines new data with a comprehensive review on GPIV.

Early literature on the subject, largely forgotten since, but well presented here, led to the belief that genetic variants of GPVI had an ethnic distribution with most cases in the Middle East.

Up to now, the variants have been largely asymptomatic but leading to alloantibody formation due to incompatibility after transfusion or pregnancy.

As well described in this article, GPIV has many functions and is widely distributed among cell types with both hemostatic (receptor for thrombospondin, scavenging receptor) and non-hemostatic roles (e.g. lipid metabolism, bone formation).

The gene is highly polymorphic and the disease-causing variants may be homozygous or heterozygous; a resulting GPVI deficiency is variable with both type I and type II disease described.

What is interesting, is that Loredana Bury and her colleagues are the first to describe mucocutaneous bleeding associated with GPIV variants and this changes the picture.

One of the cases also had bone-related problems. Intriguingly, platelet function testing was basically normal except for a reduced thrombus formation on collagen under flow (confirming early reports) and a prolonged PFA-100 closure time.

All four patients associated heterozygous mutations with a much-reduced GP IV expression in platelets (10-20 percent) and a 50 percent expression in monocytes.

Two of the variants were detected by whole genome sequencing.

How do the authors explain such a large reduction for a heterozygous expression?

And what are the molecular consequences of such a large deficit?

I will let you read their excellent discussion… This is a remarkable paper that relances the interest in GPIV and, dare I say it, in thrombospondin that too has largely been forgotten.

Highly recommended.”

Title: Inherited Platelet GPIV Deficiency: First Description of a Series of Unrelated Patients with Bleeding Diathesis

Authors: Loredana Bury, Silvia Sorrentino, Emanuela Falcinelli, Giuseppe Guglielmini, Antonietta Ferretti, Paola Concolino, Ana Sánchez-Fuentes, José Rivera, Paolo Gresele, Erica De Candia

Alan Nurden: Inherited GPIV (CD36) Deficiency Linked to Bleeding for the First Time

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Alan Nurden Ana Sánchez-Fuentes Antonietta Ferretti Biomolecules bleeding diathesis Emanuela Falcinelli Erica De Candia Giuseppe Guglielmini GPIV Health Hematology Hemostasis Hemostasis News Hemostasis Today José Rivera Medicine Paola Concolino Paolo Gresele Silvia Sorrentino
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