OBJECTIVE: To platelet aggregometry and describe the clinical spectrum of Glanzmanns thrombasthenia diagnosed by platelet aggregometry. STUDY DESIGN: A case-series. PLACE AND DURATION OF STUDY: This study was carried out at the clinical laboratories at the Aga Khan University Hospital, Karachi from January 2003 to January 2006. PATIENTS AND METHODS: All patients irrespective of age and gender presenting with bleeding symptoms and having normal platelet count were evaluated. Demographic details, relevant clinical history along with results of complete blood count, bleeding time and platelet aggregation studies were retrieved through computerized data base and evaluated for the diagnosis of Glanzmanns thrombasthenia. RESULTS: During the study period, 50 out of 2317 patients (2.2%) were diagnosed as Glanzmanns thrombasthenia by platelet aggregometry with male to female ratio of 0.85:1 and median age of 10.2 years (ranging from 3 months to 27 years). Common symptoms were epistaxis, oral and gingival

Glanzmanns thrombasthenia is an abnormality of the platelets. It is an extremely rare coagulopathy (bleeding disorder due to a blood abnormality), in which the platelets contain defective or low levels of glycoprotein IIb/IIIa (GpIIb/IIIa), which is a receptor for fibrinogen. As a result, no fibrinogen bridging of platelets to other platelets can occur, and the bleeding time is significantly prolonged. Characteristically, there is increased mucosal bleeding: menorrhagia easy bruising epistaxis gingival bleeding gastrointestinal bleeding postpartum bleeding increased bleeding post-operatively. The bleeding tendency is variable but may be severe. Hemarthrosis, particularly spontaneous, is very rare, in contrast to the hemophilias. Platelet numbers and morphology are normal. Platelet aggregation is normal with ristocetin, but impaired with other agonists such as ADP, thrombin, collagen or epinephrine. Glanzmanns thrombasthenia can be inherited in an autosomal recessive manner or acquired as an ...

Glanzmann thrombasthenia results from a deficiency of the GP IIb/IIIa complex. Platelets do not aggregate to any agents except ristocetin. The more severe type I results from a complete absence of the... more

Glanzmann thrombasthenia is a genetic platelet disorder in which the platelet have qualitative or quantitative deficiencies of the fibrinogen receptor αIIbβ3. The genes of both of these proteins are on chromosome 17, and 50% activity of each protein is enough to support normal platelet aggregation.

Laboratory tests are necessary to diagnose GT.. In people with GT, the platelet count is normal. The platelets have a normal size and shape when viewed under a microscope.. A person with GT will usually have a long bleeding time. Their results with the PFA 100® will also be abnormal ...

TY - JOUR. T1 - Association of Factor V Secretion with Protein Kinase B Signaling in Platelets from Horses with Atypical Equine Thrombasthenia. AU - Norris, J. W.. AU - Pombo, M.. AU - Shirley, E.. AU - Blevins, G.. AU - Tablin, Fern. PY - 2015/9/1. Y1 - 2015/9/1. N2 - Background: Two congenital bleeding diatheses have been identified in Thoroughbred horses: Glanzmann thrombasthenia (GT) and a second, novel diathesis associated with abnormal platelet function in response to collagen and thrombin stimulation. Hypothesis/Objectives: Platelet dysfunction in horses with this second thrombasthenia results from a secretory defect. Animals: Two affected and 6 clinically normal horses. Methods: Ex vivo study. Washed platelets were examined for (1) expression of the αIIb-β3 integrin; (2) fibrinogen binding capacity in response to ADP and thrombin; (3) secretion of dense and α-granules; (4) activation of the mammalian target of rapamycin (mTOR)-protein kinase B (AKT) signaling pathway; and (5) cellular ...

KRDS (Lys-Arg-Asp-Ser), a tetrapeptide from human lactotransferrin, was tested in vitro on human platelet function, and its effects were compared to those of RGDS, a tetrapeptide from human fibrinogen. Both peptides had a high probability of initiating a beta-turn and were highly hydrophilic. KRDS inhibited ADP-induced platelet aggregation [median inhibitory concentration (IC50) 350 microM] and fibrinogen binding (IC50 360 microM) to a lesser extent than RGDS (IC50 75 microM and 20 microM, respectively). Different from RGDS, thrombin-induced serotonin release was inhibited by KRDS (750 microM) on normal platelets (55 +/- 10%) and type I Glanzmanns thrombasthenia platelets (43% +/- 1). However, KRDS had no effect on cytoplasmic Ca2+ mobilization, inositol phospholipid metabolism or protein phosphorylation (myosin light chain P20 and P43). In contrast to RGDS, KRDS does not inhibit the binding of monoclonal antibody PAC-1 to activated platelets. KRDS and RGDS inhibited 4 beta-phorbol-12-myristate-13

If there is clinical suspicion for GT, it is reasonable to rule out other causes of mucocutaneous bleeding with a Prothrombin time (PT), Partial thromboplastin time (PTT), complete blood count (CBC), fibrinogen activity, and von Willebrand disease panel (ristocetin cofactor assay, VWF antigen, FVIII activity), all of which will be normal. In addition, platelet morphology and quantity will be normal with GT. Utilizing the platelet function analyzer (PFA-100) to screen for GT is reasonable, as nearly all cases will have a significantly prolonged closure time.. To verify the diagnosis, light transmission platelet aggregometry should be done to demonstrate the absent response to all standard platelet agonists (e.g., arachidonic acid, adenosine diphosphate (ADP), collagen, epinephrine). There is a primary wave response to ristocetin but no secondary wave response, indicating an inability to aggregate.. Definitive diagnosis can be made by defective or absent Glycoprotein expression of IIb/IIIa by flow ...

The adhesion receptors known as integrins perform key functions for hematopoietic cells. The platelet integrin αIIbβ3 is critical in hemostasis, and the β1 and β2 integrins on leukocytes have many roles in cell-mediated immunity. Mutations in the β2 subunit lead to integrin nonexpression and to an immune deficiency, leukocyte adhesion deficiency-1. Mutations in either the α or β subunit of αIIbβ3 usually lead to integrin nonexpression and a bleeding tendency termed Glanzmann thrombasthenia. Here we describe a unique patient with clinical features of both Glanzmann thrombasthenia and leukocyte adhesion deficiency-1. The patient has normal expression of β1, β2, and β3 integrins, but all are dysfunctional. The key findings are that inside-out signaling pathways leading to integrin activation are defective and that this is associated with abnormal integrin clustering. The integrins themselves are intact and capable of function following extracellular stimulation. T cell motility is ...

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TROMBASTENIA DE GLANZMANN PDF - 29 Dec Glanzmann thrombasthenia is a genetic platelet disorder in which the platelet have qualitative or quantitative deficiencies of the fibrinogen.

2012 (English)In: Haemophilia, ISSN 1351-8216, E-ISSN 1365-2516, Vol. 18, no 5, e381-e383 p.Article in journal, Letter (Refereed) Published ...

ENFERMEDAD DE GLANZMANN PDF - Cristian «Chrigel» Glanzmann (nacido como Christian Oliver Ivan Glanzmann en en Basilea, Suiza) es el vocalista de la banda de folk metal Eluveitie.

PMID 9920835] Molecular genetic analysis of a compound heterozygote for the glycoprotein (GP) IIb gene associated with Glanzmanns thrombasthenia: disruption of the 674-687 disulfide bridge in GPIIb prevents surface exposure of GPIIb-IIIa complexes. ...

CD61, also called integrin beta-3, belongs to the integrin protein family that participates in cell adhesion and cell-surface mediated signalling by forming heterodimers of alpha and beta integrin chains. Integrin alpha-V/beta-3 is a receptor for cytoactin, fibronectin, laminin, matrix metalloproteinase-2, osteopontin, osteomodulin, prothrombin, thrombospondin, vitronectin and von Willebrand factor. Integrin alpha-IIb/beta-3 is a receptor for fibronectin, fibrinogen, plasminogen, prothrombin, thrombospondin and vitronectin. These two integrins recognize the sequence RGD in a wide array of ligands. Integrin IIb/IIIa recognizes the sequence HHLGGGAKQAGDV in fibrinogen gamma chain. Following activation, integrin alpha-IIb/beta-3 supports platelet/platelet interaction through binding of soluble fibrinogen, leading to rapid platelet aggregation which physically plugs ruptured endothelial surface. Defects in CD61 are a cause of Glanzmann thrombasthenia, a blot clot disorder ...

Hello,Glanzmann thrombasthenia is also known as weak platelet disease.Platelets are small cells which help to control bleeding.In this rare condition affecting iraqi,and arab descent population, leads individual platelets unable to aggregate plug a bleeding area even though they may be present in adequate numbers.Minor injuries can lead to bleeding like of gums,nose and in females, during menstruation excessive bleeding ...

These integrin beta 3 targeted mutant mice exhibit significant embryonic lethality attributed to fetal hemorrhaging and placental defects. This mutant mouse strain represents a model that may be useful in studies related to Glanzmann thrombasthenia.

These integrin beta 3 targeted mutant mice exhibit significant embryonic lethality attributed to fetal hemorrhaging and placental defects. This mutant mouse strain represents a model that may be useful in studies related to Glanzmann thrombasthenia.

Korfel, A; Thiel, E; Martus, P; Möhle, R; Griesinger, F; Rauch, Mi; Röth, A; Hertenstein, B; Fischer, T; Hundsberger, T; Mergenthaler, H G; Junghanß, C; Birnbaum, T; Fischer, L; Jahnke, K; Herrlinger, U; Roth, P; Bamberg, M; Pietsch, T; Weller, M (2015). Randomized phase III study of whole-brain radiotherapy for primary CNS lymphoma. Neurology, 84(12):1242-1248.. Sandrock-Lang, K; Oldenburg, J; Wiegering, V; Halimeh, S; Santoso, S; Kurnik, K; Fischer, L; Tsakiris, D A; Sigl-Kraetzig, M; Brand, B; Bührlen, M; Kraetzer, K; Deeg, N; Hund, M; Busse, E; Kahle, A; Zieger, B (2015). Characterisation of patients with Glanzmann thrombasthenia and identification of 17 novel mutations. Thrombosis and Haemostasis, 113(4):782-791.. Huggel, C; Allen, S; Deline, P; Fischer, L; Noetzli, J; Ravanel, L (2012). Ice thawing, mountains falling-are alpine rock slope failures increasing? Geology Today, 28(3):98-104.. Benz, K; Stippich, C; Fischer, L; Möhl, K; Weber, K; Lang, J; Steffen, F; Beintner, B; ...

Clone REA386 recognizes the human CD41a antigen, a calcium-dependent complex of CD41/CD61 (GPIIb/IIIa) expressed on normal platelets and megakaryocytes. The CD41/CD61 complex is a receptor for fibronectin, fibrinogen, von Willebrand factor, vitronectin, and thrombospondin and is required for platelet aggregation and clotting. Mutations of either CD41 or CD61 in humans causes Glanzmanns thrombasthenia. Notably, CD41 is also the first blood-specific marker during in vitro differentiation of embryonic stem cells and is expressed on all hematopoietic stem and progenitor cells in the early embryo. However, CD41 expression is downregulated in midgestation embryos, and it is currently thought that CD41 expression on adult hematopoietic stem cells remains switched off throughout adult life. Additional information: Clone REA386 displays negligible binding to Fc receptors. - Deutschland

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TY - JOUR. T1 - Two novel mutations in the αIIb calcium-binding domains identify hydrophobic regions essential for αIIbβ3 biogenesis. AU - Mitchell, W. Beau. AU - Li, Ji Hong. AU - Singh, Fiza. AU - Michelson, Alan D.. AU - Bussel, James. AU - Coller, Barry S.. AU - French, Deborah L.. N1 - Copyright: Copyright 2008 Elsevier B.V., All rights reserved.. PY - 2003/3/15. Y1 - 2003/3/15. N2 - The recently published crystal structure of the external domains of αVβ3 confirms the prediction that the aminoterminal portion of αV, which shares 40% homology with αIIb, folds into a β-propeller structure and that the 4 calcium-binding domains are positioned on the bottom of the propeller. To gain insight into the role of the calcium-binding domains in αIIb biogenesis, we characterized mutations in the second and third calcium-binding domains of αIIb in 2 patients with Glanzmann thrombasthenia. One patient inherited a Val298Phe mutation in the second domain, and the other patient inherited an ...

Bacon gets all the internet glory, but its more old-fashioned cousin salt pork may actually be good for you-for your nosebleeds, if not your waistline. Doctors recently used strips of cured salt pork to stop a life-threatening nosebleed. One of the doctors remembered the unconventional treatment from a field manual he saw in his military days, after exhausting all medical treatments short of risky surgeries.. The patient was a four-year-old girl with Glanzmann thrombasthenia, a rare blood disorder where her platelets are unable to do their normal job of blood clotting. Surgery and injection of blood coagulation proteins didnt stop her bleeding after more than a week, so the doctors turned to something untested and low tech: Cured salted pork crafted as a nasal tampon and packed within the nasal vaults successfully stopped nasal hemorrhage promptly, effectively, and without sequelae, they wrote in a paper about the episode. While nasal tampon may sound distinctly undelicious as a pork ...

TY - JOUR. T1 - Demonstration of novel gain-of-function mutations of αIIbβ3. T2 - Association with macrothrombocytopenia and glanzmann thrombasthenia-like phenotype. AU - Kashiwagi, Hirokazu. AU - Kunishima, Shinji. AU - Kiyomizu, Kazunobu. AU - Amano, Yoshiro. AU - Shimada, Hiroyuki. AU - Morishita, Masashi. AU - Kanakura, Yuzuru. AU - Tomiyama, Yoshiaki. N1 - Publisher Copyright: © 2013 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.. PY - 2013/7. Y1 - 2013/7. N2 - Integrin aIIbb3 is indispensable for normal hemostasis, but its role for thrombopoiesisis still controversial. Recently, αIIb and β3 mutations have been identified in patients with congenital macrothrombocytopenia. We analyzed three unrelated Japanese families with congenital macrothrombocytopenia. Expression and activation state of αIIbβ3 in platelets was examined by flow cytometry and immunoblotting. Sequence of whole coding region and exon-intron boundariesof ITGA2B and ITGB3 genes ...

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CHAPTER 119 HEREDITARY QUALITATIVE PLATELET DISORDERS Williams Hematology CHAPTER 119 HEREDITARY QUALITATIVE PLATELET DISORDERS BARRY S. COLLER DEBORAH L. FRENCH Glycoprotein Abnormalities Glycoprotein IIb/IIIa (aIIbb3; CD41/CD61) - Glanzmann Thrombasthenia Glycoprotein Ib (CD42b,c), IX (CD42a), and V-Bernard-Soulier Syndrome GPIb (CD42b,c)-Platelet-Type (Pseudo-) von Willebrand Disease Glycoprotein Ia/IIa (a2b1; VLA-2; CD49b/CD29) GPIV (CD36) GPVI Abnormal Membrane-Cytoskeletal Interactions Wiskott-Aldrich…

Activated blood platelets mediate the primary response to vascular injury. Although molecular abnormalities of platelet proteins occur infrequently, taken collectively, an inherited platelet defect accounts for a bleeding diathesis in ≈1:20,000 individuals. One rare example of a platelet disorder, Glanzmann thrombasthenia (GT), is characterized by life-long morbidity and mortality due to molecular abnormalities in a major platelet adhesion receptor, integrin αIIbβ3. Transfusion therapy is frequently inadequate because patients often generate antibodies to αIIbβ3, leading to immunemediated destruction of healthy platelets. In the most severe cases allogeneic bone marrow transplantation has been used, yet because of the risk of the procedure it has been limited to few patients. Thus, hematopoietic stem cell gene transfer was explored as a strategy to improve platelet function within a canine model for GT. Bleeding complications necessitated the use of a mild pretransplant conditioning ...

Rarely Autosomal Dominant Inheritance Symptom Checker: Possible causes include Glanzmann Thrombasthenia & Peutz-Jeghers Syndrome & Quebec Platelet Disorder. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search.

ALBLD : Detection of the more common potential causes of abnormal bleeding (eg, factor deficiencies/hemophilia, von Willebrand disease, factor-specific inhibitors) and a simple screen to evaluate for an inhibitor or severe deficiency of factor XIII (rare)   This test is not useful for assessing platelet function (eg, congenital or acquired disorders such as Glanzmann thrombasthenia, Bernard-Soulier syndrome, storage pool disease, myeloproliferative disease, associated platelet dysfunction), which requires fresh platelets

Integrin alpha-IIb is a protein that in humans is encoded by the ITGA2B gene. ITGA2B, also known as CD41, encodes integrin alpha chain 2b. Integrins are heterodimeric integral membrane proteins composed of an alpha chain and a beta chain. Alpha chain 2b undergoes post-translational cleavage to yield disulfide-linked light and heavy chains that join with beta 3 to form a fibrinogen receptor expressed in platelets that plays a crucial role in coagulation. Mutations that interfere with this role result in thrombasthenia. In addition to adhesion, integrins are known to participate in cell-surface mediated signalling.[5] ...

InterPro provides functional analysis of proteins by classifying them into families and predicting domains and important sites. We combine protein signatures from a number of member databases into a single searchable resource, capitalising on their individual strengths to produce a powerful integrated database and diagnostic tool.

The effect of genetic variants on aspirin resistance (AR) remains controversial. We sought to assess the association of genetic variants with AR and early clinical outcomes in patients with acute ischemic stroke (IS). A total of 850 acute IS patients were consecutively enrolled. Platelet aggregation was measured before and after a 7-10 day aspirin treatment. The sequences of 14 variants of COX-1, COX-2, GPIb, GPIIIa, P2Y1 and P2Y12 were determined using matrix-assisted laser desorption/ionization time of flight mass spectrometry. Gene-gene interactions were analyzed using generalized multifactor dimensionality reduction (GMDR). The primary outcome was early neurological deterioration (END) within 10 days of admission. The secondary outcome was a composite of early recurrent ischemic stroke (ERIS), myocardial infarction (MI) and death within 10 days of admission. 175 (20.6%) patients were AR, 45 (5.3%) were aspirin semi-resistant, 121 (14.2%) developed END, 17 (0.2%) had ERIS, 2 (0.2%) died, and 6 (0.7%)

Platelets, or thrombocytes, are very small, irregularly shaped clear cell fragments, 2-3 µm in diameter, which derive from fragmentation of megakaryocytes. The average lifespan of a platelet is normally just 5 to 9 days. Platelets are a natural source of growth factors. They circulate in the blood of mammals and are involved in hemostasis, leading to the formation of blood clots. Platelets release thread-like fibers to form these clots. If the number of platelets is too low, excessive bleeding can occur. However, if the number of platelets is too high, blood clots can form thrombosis, which may obstruct blood vessels and result in such events as a stroke, myocardial infarction, pulmonary embolism-or blockage of blood vessels to other parts of the body, such as the extremities of the arms or legs. An abnormality or disease of the platelets is called a thrombocytopathy, which can be either a low number of platelets (thrombocytopenia), a decrease in function of platelets (thrombasthenia), or an ...

The department is engaged in active research with investigations ranging from a basic to molecular level in the field leukemia, bleeding disorders, platelet function disorders, hemoglobinopathies, thalassemia etc. The department awards PhD degrees to postgraduates in the field of Biochemistry, Genetics and Biotechnology. The research workers have made a significant contribution to the Department by introducing, standardizing and monitoring the various modern molecular tests. They have contributed in the establishment of tests such as Prenatal diagnosis of Hemophilia, Multimeric analysis of vWD, screening for Thrombophilic mutations,estimation of gpIIb/IIIa in Glanzmanns Thrombasthenia, alpha3.7 and 4.2 deletions in Thalassemia, immunophenotypic and molecular studies (RTPCR) for Cytogenetics in diagnosis and management of haematological malignancies such as bcr/abl for chronic myeloid leukemia. Most of these tests are either not available or done at only select centers across the country. The ...

February 11, 2016 -- While major adverse events from liver biopsy guided by ultrasound or CT are extremely rare, variables such as the patients platelet count can increase the risk for hematoma from the procedure by as much as fourfold, according to a large retrospective analysis performed at the Mayo Clinic in Rochester, MN ...

Translational perspective Genetic deletion of p66Shc, as shown in the present study, leads to increased myocardial infarction in response to short-term ischaemia and reperfusion. [...]heart-specific ... European Heart Journal, BASIC SCIENCE, Alexander Akhmedov, Fabrizio Montecucco, Vincent Braunersreuther, Giovanni G. Camici, Philipp Jakob, Martin F. Reiner, Martina Glanzmann, Fabienne Burger, ... ...

L eptacog alpha ou rFVIIa (NovoSeven ) a t commercialis en 1996 dans le traitement des h morragies de l h mophilie cong nitale avec inhibiteur puis en 1998 dans le traitement des syndromes h morragiques de l h mophilie acquise. Des extensions d indications ont t obtenues en 2004 dans le traitement des manifestations h morragiques survenant dans la thrombasth nie de Glanzmann avec anticorps antiGPIIb-IIIa et/ou anti-HLA, le d ficit constitutionnel en FVII. En raison de son mode d action original et d une action h mostatique ind niable, l eptacog alpha a t utilis dans de nombreuses situations cliniques. Cependant, dans ces indications, l valuation clinique est encore souvent insuffisante.. En cas de l sions vasculaires, l h mostase primaire et la coagulation interviennent simultan ment. L h mostase primaire repr sente l ensemble des interactions entre la paroi vasculaire, les plaquettes et le facteur Willebrand qui aboutit l obturation de la br che vasculaire par un thrombus blanc. La coagulation ...

Chapter Outline QUALITATIVE DISORDERS Platelet Membrane Glanzmann Thrombasthenia: Defective Platelet Integrin α IIb β 3 Other Integrins in Inherited Platelet Disorders Bernard-Soulier Syndrome: Defective Glycoprotein Ib/IX Complex Platelet-Type von Willebrand Disease (Pseudo-von Willibrand Disease) Other Inherited Defects of Platelet Receptors Defects in Signal Transduction Platelet Storage Granule Defects QUANTITATIVE DISORDERS Disorders of Platelet Production Platelets…

Congenital disorders of platelet function are a heterogeneous group of disorders that are often not detected until bleeding occurs. In clinical settings only a few methods have proven to be useful for identification and classification of inherited platelet disorders. For a rational diagnostic approach, a stepwise algorithm is recommended. Patient history and clinical investigation are mandatory. Von Willebrand disease and other coagulation disorders should always be ruled out prior to specific platelet testing. Platelet count, size, volume (MPV) and morphology may guide further investigations. The PFA-100® CT is suited for screening for severe platelet defects. Platelet aggregometry allows assessment of multiple aspects of platelet function. Flow cytometry enables diagnosis of thrombasthenia Glanzmann, Bernard-Soulier syndrome and storage pool defects. Molecular genetics may confirm a putative diagnosis or pave the way for identifying new defects. We present an unabridged version of the ...

Normal primary platelet aggregation requires agonist-mediated activation of membrane GPIIb-IIIa, binding of fibrinogen to GPIIb-IIIa, and cellular events after ligand binding. PAC1 monoclonal antibody distinguishes between resting and activated states of GPIIb-IIIa, and other antibodies preferentially recognize GPIIb (PMI-1) or IIIa (anti-LIBS1) after the binding of fibrinogen or fibrinogen-mimetic peptides, such as GRGDSP. Using these antibodies and platelet flow cytometry, we studied two distinct persistent platelet aggregation abnormalities. Platelets from a thrombasthenic variant, which contained near-normal amounts of GPIIb-IIIa, failed to aggregate or bind PAC1 in response to agonists. In addition, GRGDSP, which binds to normal GPIIb-IIIa without prior cell activation, failed to increase the binding of PMI-1 or anti-LIBS1 to the thrombasthenic platelets, suggesting a primary defect in ligand binding. Chromatography of detergent-solubilized platelets on a KYGRGDS affinity column confirmed ...

To delineate the critical top features of platelets necessary for balance and formation of thrombi, thromboelastography and platelet aggregation measurements were employed in whole blood of normal individuals and of those with Bernard-Soulier Syndrome (BSS) and Glanzmanns Thrombasthenia (GT). generation of stable thrombi, a potentially significant feature in individual medical results. Introduction An initial step in thrombus formation in the hurt vascular endothelium is the adhesion of platelets to shown subendothelial elements, e.g., von Willebrand Aspect (vWF), under high prices of shear, via the connections from the platelet glycoprotein (GP) 1b/V/IX receptor complicated with subendothelial vWF [1]. This tethering of platelets after that promotes their firmer binding to subendothelial collagen (COL) fibres via platelet receptors, e.g., GPVI [2], [3] and integrin II1 [4]. In this procedure, platelets are turned on, resulting in platelet shape adjustments, aggregation, discharge of aggregation ...

TY - JOUR. T1 - Effects of ATP on ligand recognition of platelet fibrinogen receptor on GPIIb-IIIa. AU - Gawaz, M. P.. AU - Mayinger, P.. AU - Neumann, F. J.. PY - 1994. Y1 - 1994. N2 - The recent discovery of 8-azido-ATP binding sites on the platelet fibrinogen receptor glycoprotein complex GPIIb-IIIa suggests that extracellular ATP may directly modulate function of GPIIb-IIIa. In this study we investigated the effect of ATP on ligand binding to GPIIb-IIIa. Fibrinogen-mediated aggregation of washed platelets was inhibited by ATP and 8-azido-ATP in a dose-dependent manner, independent of the agonist (thrombin, collagen, epinephrine, phorbol 12-myristate 13-acetate) used to induce platelet activation. In addition, 8-azido-ATP and ATP inhibited binding of 125I-labeled fibrinogen to thrombin- and phorbol ester-activated platelets. Interaction of nonstimulated platelets with solid-phase fibrinogen was also reduced by 8-azido-ATP and ATP. Moreover, fibrinogen mimetic peptide-induced conformational ...

MalaCards based summary : Hypothyroidism, Congenital, Nongoitrous, 6, also known as chng6, is related to thrombasthenia and retinal ischemia, and has symptoms including dry skin and constipation. An important gene associated with Hypothyroidism, Congenital, Nongoitrous, 6 is THRA (Thyroid Hormone Receptor Alpha), and among its related pathways/superpathways are Colorectal Cancer Metastasis and 14-3-3 and Regulation of BAD Activity. Affiliated tissues include thyroid, liver and pituitary, and related phenotypes are macroglossia and delayed skeletal maturation ...

Other coagulation screening tests: Activated clotting time: • measures time for clot to form (especially during heparin therapy) Clot retraction time: • studies function of platelets in clot formation (especially in Glanzmanns disease) Coagulation factor assay: • used to detect the deficiency o ...

Reactome is pathway database which provides intuitive bioinformatics tools for the visualisation, interpretation and analysis of pathway knowledge.

lysyl-tyrosyl-glycyl-cysteinyl-homoarginyl-glycyl-aspartyl-tryptophyl-prolyl-cysteine cyclic (4-10)-disulfide: cHarGD - cyclic homoarginine-glycine-aspartic acid-containing peptide; ligand for platelet membrane glycoprotein IIb-IIIa

Platelet aggregation requires the binding of fibrinogen to its receptor, a heterodimer consisting of the plasma-membrane glycoproteins (GP) IIb and IIIa. Although the GPIIb-IIIa complex is present on the surface of unstimulated platelets, it binds fibrinogen only after platelet activation. We have used an immunogold-surface replica technique to study the distribution of GPIIb-IIIa and bound fibrinogen over broad areas of surface membranes in unstimulated, as well as thrombin-activated and ADP-activated human platelets. We found that the immunogold-labeled GPIIb-IIIa was monodispersed over the surface of unstimulated platelets, although the cell surface lacked immunoreactive fibrinogen. On thrombin-stimulated platelets, approximately 65% of the GPIIb-IIIa molecules were in clusters within the plane of the membrane. Fibrinogen, which had been released from the alpha-granules of these cells, bound to GPIIb-IIIa on the cell surface and was similarly clustered. To determine whether the receptors ...

New Delhi: According to International guidelines, unless a patients platelet count is below 10,000, and there is spontaneous, active bleeding... Dengue

Literature References: Platelet fibrinogen receptor (GPIIb/IIIa) antagonist. Prodrug converted in vivo to the active acid form. Prepn: P. R. Bovy et al., WO 9307867; eidem, US 5344957 (1993, 1994 both to Monsanto; Searle); J. Cossy et al., Bioorg. Med. Chem. Lett. 7, 1699 (1997). Structure-activity study: J. A. Zablocki et al., J. Med. Chem. 38, 2378 (1995). Pharmacology: N. S. Nicholson et al., Circulation 91, 403 (1995). Clinical pharmacokinetics: D. J. Kereiakes et al., ibid. 94, 906 (1996). Clinical evaluation in unstable angina: C. Simpfendorfer et al., ibid. 96, 76 (1997). ...

The inherited platelet disorders are an uncommon cause of symptomatic bleeding. They may be difficult to diagnose (and are likely to be under-diagnosed) and pose problems in management. This review discusses the inherited platelet disorders summarising the current state of the art with respect to in …

3-methylglutaconic aciduria (3-MGA) is considered a rare condition. Less than 20 cases of MGA type I have been reported. 3MGA type II is estimated to affect one out of every 200,000 males. MGA type III is extremely rare in most populations. It is more common in the Iraqi-Jewish population where it affects approximately one in every 10,000 individuals. Exact numbers of affected individuals with MGA type IV and MGA type V are currently not available. MGA type V has only been reported in the Hutterite population of North America and Canada ...