PubMed HealthSearch

PubMed · 2931803

Platelet membrane proteins.

Abstract

Studies on platelet membrane proteins during the past decade have defined a series of such components and their roles in key phenomena in hemostasis and thrombosis such as platelet adhesion and aggregation. Central among the membrane proteins is the glycoprotein group. At this time, several of these proteins have been characterized as to apparent molecular weights and subunit composition whereas sugar and amino acid composition and sequences await further studies for most of them. The absence of specific membrane glycoproteins in congenital bleeding diseases has helped to identify their functions as receptors for known ligands for binding reactions central to the hemostatic mechanism. Some of the membrane proteins may exist more or less loosely attached to each other on the surface of intact platelets, and this may be important for their functions.

Explore related subjects

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

N O Solum. 1985. Platelet membrane proteins.. https://pubmed.ncbi.nlm.nih.gov/2931803/

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

Molecular characterization of two mutations in platelet glycoprotein (GP) Ib alpha in two Finnish Bernard-Soulier syndrome families.

Bernard-Soulier syndrome (BSS) is a rare hereditary bleeding disorder and macrothrombocytopenia which is caused by a defect in the platelet glycoprotein Ib/IX/V (GP Ib/IX/V) complex, the receptor for von Willebrand factor and thrombin. Here we report the molecular basis of the classical form of BSS in two unrelated Finnish patients, both with a life-long history of severe bleeding. Flow cytometry and immunoblotting showed no expression of GP Ib/IX, GP Ib alpha, GP Ib beta or GP IX (less than 10%) in the patients' platelets. No expression of GP V (< 10%) was observed in propositus 1, but a residual amount was found in propositus 2 (24%). DNA sequencing analysis revealed that propositus 1 was compound heterozygous for a two-base-pair deletion at Tyr505(TAT) and a point mutation Leu129(CTC)Pro(CCC) in the GP Ib alpha gene. Propositus 2 was homozygous for the Tyr505(TAT) deletion. The nine relatives who were heterozygous for either of the mutations also had low levels of GP Ib alpha (74-90%). Hence, Bernard-Soulier patients homozygous or compound heterozygous for Tyr505(TAT) are severely affected. Interestingly, both mutations have independently been found in three other families in previous reports, suggesting their ancient age or mutational 'hot spot'.

Bernard-Soulier Syndrome

Use of the electron microscope for diagnosis of platelet disorders.

The electron microscope is generally regarded as a sophisticated instrument used almost exclusively for basic research. However, ultrastructural methods can be just as valuable for the clinical diagnosis of inherited platelet disorders, as for more fundamental studies. This report describes several instances in which electron microscopy has been critical for identifying and characterizing genetic problems. For example, platelet storage pool deficiency (SPD) is due to a marked decrease or absence of the organelles storing adenine nucleotides, serotonin, and calcium destined for secretion during the platelet release reaction. The organelles are referred to as dense bodies because they are inherently electron opaque. As a result, platelet SPD is more rapidly and reliably diagnosed in the electron microscope than by any other technique. Giant platelet disorders have presented a bewildering array. The electron microscope has made it possible to separate the various types into distinct conditions based on the nature of platelet structural defects and inclusions found in leukocytes. Immunogold and cytochemical techniques have expanded the horizon of electron microscopy in the evaluation of platelet disorders and have assured its continued use for this purpose in the future.

Bernard-Soulier Syndrome