Professor, Pediatrics, Perelman School of Medicine
Pediatric Hematology & Oncology
Dr. Mortimer Poncz, MD
|
|
Give Review
Dr. Mortimer Poncz is an expert in the field of Pediatric Hematology & Oncology. He went to medical school at Perelman School of Medicine at the University of Pennsylvania. He has 2 awards "Elected Member" and "Super Doctor". Doctor Mortimer Poncz, MD is a published doctor. He has 101 papers published. The most current publication "Defining an antigenic epitope on platelet factor 4 associated with heparin-induced thrombocytopenia."
Publications
- CRISPR/Cas9-mediated conversion of human Platelet alloantigen allotypes.
- Molecular engineering of high affinity single-chain antibody fragment for endothelial targeting of proteins and nanocarriers in rodents and humans.
- Platelet transactivation by monocytes promotes the prothrombotic State in heparin-induced thrombocytopenia.
- Intramedullary megakaryocytes internalize released Platelet factor 4 (PF4) and store it in alpha granules.
- Platelet-delivered ADAMTS13 inhibits arterial Thrombosis and prevents murine models of thrombotic thrombocytopenic purpura.
- Apoptotic effects of Platelet factor VIII on megakaryopoiesis: implications for a modified human FVIII for Platelet-based gene therapy.
- Defective release of α-granule and lysosome contents from Platelets in mouse Hermansky-Pudlak syndrome models.
- Megakaryocytes regulate hematopoietic stem Cell quiescence through CXCL4 secretion.
- T2 Magnetic Resonance: A Diagnostic Platform for Studying Integrated Hemostasis in Whole Blood-Proof of Concept.
- Personalized Platelet transfusions: one step closer to the clinic.
- Atherosclerosis is not a risk factor for anti-Platelet factor 4/heparin antibody formation after cardiopulmonary bypass surgery.
- High-level transgene expression in induced pluripotent stem cell-derived megakaryocytes: correction of Glanzmann Thrombasthenia.
- They're not your daddy's inherited platelet Disorders anymore.
- Modulation of Protein C Activation by Histones, Platelet Factor 4, and Heparinoids: New Insights Into Activated Protein C Formation.
- Minimal modification in the factor VIII B domain sequence ameliorates the murine Hemophilia A phenotype.
- Challenges and promises for the development of donor-independent Platelet transfusions.
- Cooperative integrin/ITAM signaling in Platelets enhances thrombus formation in vitro and in vivo.
- Platelet factor 4 platelet levels are inversely correlated with steady-State platelet counts and with platelet transfusion needs in pediatric leukemia patients.
- SLC35D3 delivery from megakaryocyte early endosomes is required for Platelet dense granule biogenesis and differentially defective in Hermansky-Pudlak syndrome models.
- Targeting recombinant thrombomodulin fusion protein to red blood Cells provides multifaceted thromboprophylaxis.
- Heparin-induced thrombocytopenia/Thrombosis in a transgenic mouse model requires human platelet factor 4 and platelet activation through FcgammaRIIA.
- Distinct domains of alphaIIbbeta3 support different aspects of outside-in signal transduction and Platelet activation induced by LSARLAF, an alphaIIbbeta3 interacting ...
- Localization of distal regulatory domains in the megakaryocyte-specific Platelet basic protein/Platelet factor 4 gene locus.
- Heparin-induced thrombocytopenia: bovine versus porcine heparin in cardiopulmonary bypass surgery.
- RGD-containing peptides inhibit fibrinogen binding to Platelet alpha(IIb)beta3 by inducing an allosteric change in the amino-terminal portion of alpha(IIb).
- Stromal-derived factor 1 and thrombopoietin regulate distinct aspects of human megakaryopoiesis.
- The regulated expression of a TATA-less, Platelet-specific gene, alphaIIb.
- Loss of signaling through the G protein, Gz, results in abnormal Platelet activation and altered responses to psychoactive drugs.
- The alpha subunits of Gz and Gi interact with the eyes absent transcription cofactor Eya2, preventing its interaction with the six class of homeodomain-containing prot...
- Stromal cell-derived factor-1 and macrophage-derived chemokine: 2 chemokines that activate Platelets.
- Molecular basis and characterization of the hyperinsulinism/hyperammonemia syndrome: predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase gene. ...
- Familial dyserythropoietic anaemia and thrombocytopenia due to an inherited mutation in GATA1.
- Characterization of a murine monoclonal antibody that mimics heparin-induced thrombocytopenia antibodies.
- A naturally occurring mutation near the amino terminus of alphaIIb defines a new region involved in ligand binding to alphaIIbbeta3.
- Characterization of the murine Platelet alphaIIb gene and encoded cDNA.
- Haploinsufficiency of CBFA2 causes familial thrombocytopenia with propensity to develop acute myelogenous leukaemia.
- The human Platelet alphaIIb gene is not closely linked to its integrin partner beta3.
- Megakaryocyte precursors, megakaryocytes and Platelets express the HIV co-receptor CXCR4 on their surface: determination of response to stromal-derived factor-1 by meg...
- Defining an antigenic epitope on Platelet factor 4 associated with heparin-induced thrombocytopenia.
- Evidence for genetic homogeneity in a familial Platelet disorder with predisposition to acute myelogenous leukemia (FPD/AML)
- Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene.
- An Sp1-binding silencer element is a critical negative regulator of the megakaryocyte-specific alphaIIb gene.
- A Leu117-->Trp mutation within the RGD-peptide cross-linking region of beta3 results in Glanzmann thrombasthenia by preventing alphaIIb beta3 export to the Platelet su...
- Activation of the megakaryocyte-specific gene Platelet basic protein (PBP) by the Ets family factor PU.1.
- Prevalence of heparin-associated antibodies without Thrombosis in patients undergoing cardiopulmonary bypass surgery.
- Fc gamma RIIA H/R 131 polymorphism, subclass-specific IgG anti-heparin/Platelet factor 4 antibodies and clinical course in patients with heparin-induced thrombocytopen...
- Long-term treatment of congenital folate malabsorption.
- Rapid detection of the Fc gamma RIIA-H/R 131 ligand-binding polymorphism using an allele-specific restriction enzyme digestion (ASRED).
- Inherited mutations within the calcium-binding sites of the integrin alpha IIb subunit (Platelet glycoprotein IIb). Effects of the amino acid side chain and the amino ...
- Effect of mutagenesis of GPIIb amino acid 273 on the expression and conformation of the Platelet integrin GPIIb-IIIa.
- An Ets/Sp1 interaction in the 5'-flanking region of the megakaryocyte-specific alpha IIb gene appears to stabilize Sp1 binding and is essential for expression of this ...
- Glanzmann Thrombasthenia due to a two amino acid deletion in the fourth calcium-binding domain of alpha IIb: demonstration of the importance of calcium-binding domains...
- Presence of autoantibodies to interleukin-8 or neutrophil-activating peptide-2 in patients with heparin-associated thrombocytopenia.
- Comparison of PF4/heparin ELISA assay with the 14C-serotonin release assay in the diagnosis of heparin-induced thrombocytopenia.
- A comparative analysis of cDNA-derived sequences for rat and mouse beta 3 integrins (GPIIIA) with their human counterpart.
- Determinants of the intracellular fate of truncated forms of the Platelet glycoproteins IIb and IIIa.
- Analysis of Gz alpha by site-directed mutagenesis. Sites and specificity of protein kinase C-dependent phosphorylation.
- Glanzmann thrombasthenia secondary to a Gly273-->Asp mutation adjacent to the first calcium-binding domain of Platelet glycoprotein IIb.
- Agonist receptors and G proteins as mediators of Platelet activation.
- Characterization of regulatory elements in the 5'-flanking region of the rat GPIIb gene by studies in a primary rat marrow culture system.
- Structural requirements of Platelet chemokines for neutrophil activation.
- Platelet glycoprotein IIb gene expression as a model of megakaryocyte-specific expression.
- Chemokine regulation of human megakaryocytopoiesis.
- Agonist-stimulated ligand binding by the Platelet integrin alpha IIb beta 3 in a lymphocyte expression system.
- Congenital folate malabsorption.
- Unusual manifestations of chronic granulomatous Disease.
- beta-Thalassemia in a Kurdish Jew. Single base changes in the T-A-T-A box.
- Construction of human gene libraries from small amounts of peripheral blood: analysis of beta-like globin genes.
- Therapy of congenital folate malabsorption.
- "Nonrandom" DNA sequence analysis in bacteriophage M13 by the dideoxy chain-termination method.
- Thalassemia.
- Detection of a novel DNA polymorphism in the beta-globin gene cluster.
- Nucleotide sequence analysis of the delta beta-globin gene region in humans.
- Frequent occurrence of a zeta-globin-region deletion in American blacks accounts for a previously-described restriction site polymorphism.
- The silent carrier allele: beta Thalassemia without a mutation in the beta-globin gene or its immediate flanking regions.
- RNA synthesis by exogenous RNA polymerase on cytological preparations of chromosomes.
- Acute chest syndrome in Sickle cell disease: etiology and clinical correlates.
- Hematologic changes during acute chest syndrome in Sickle cell disease.
- Human Platelet factor 4 gene is mapped to 4q12----q21.
- DNA methylation in hereditary persistence of fetal hemoglobin (HPFH-2).
- Cloning and characterization of Platelet factor 4 cDNA derived from a human erythroleukemic cell line.
- A large deletion encompassing the entire alpha-like globin gene cluster in a family of northern European extraction.
- A Taq 1 polymorphism for the human Platelet glycoprotein IIIa gene (GP3A).
- Non-random association of the Rsa I polymorphic site 5' to the beta-globin gene with major Sickle cell haplotypes.
- An Eco R1 polymorphism of a human Platelet factor 4 (PF4) gene.
- Globin gene expression in hereditary persistence of fetal haemoglobin and (delta beta) (0)-thalassaemia.
- Structure of Platelet glycoprotein IIIa. A common subunit for two different membrane receptors.
- Comparison of cDNA-derived protein sequences of the human fibronectin and vitronectin receptor alpha-subunits and Platelet glycoprotein IIb.
- Structure of the Platelet membrane glycoprotein IIb. Homology to the alpha subunits of the vitronectin and fibronectin membrane receptors.
- The genomic organization of Platelet glycoprotein IIIa.
- Organization of the gene for Platelet glycoprotein IIb.
- Analysis of rodent Platelet glycoprotein IIb: evidence for evolutionarily conserved domains and alternative proteolytic processing.
- Molecular biology of the megakaryocyte-specific gene Platelet factor 4.
- Biologic and biochemic properties of recombinant Platelet factor 4 demonstrate identity with the native protein.
- Identification of the pertussis toxin-sensitive G proteins in Platelets, megakaryocytes, and human erythroleukemia cells.
- Polymerase chain reaction (PCR) for detection of PstI polymorphism in the human PF4 gene.
- Atypical presentation of Wiskott-Aldrich syndrome: diagnosis in two unrelated males based on studies of maternal T Cell X chromosome inactivation.
- Effect of deletion of glycoprotein IIb exon 28 on the expression of the Platelet glycoprotein IIb/IIIa complex.
- Identification of Gz alpha as a pertussis toxin-insensitive G protein in human Platelets and megakaryocytes.
- Receptor and G protein-mediated responses to thrombin in HEL Cells.
Schools
Perelman School of Medicine University of Pennsylvania
Children39s Hospital Of Philadelphia
Procedures Preformed
- HPC Transplantation (Stem Cell Transplant)
Conditions Treated
- Anemia
- Coagulation Disorders (incl. Hemophilia)
- Deep Vein Thrombosis (DVT)
- Hypercoagulable State
- View All
Doctors Specialties
- Pediatric Hematology
- Oncology
Accepted Insurances
Awards
- Elected Member
- Super Doctor
Education
-
Children's Hospital of Philadelphia -
Perelman School of Medicine at the University of Pennsylvania
Hospital
-
Hospital of the University of Pennsylvania -
Pennsylvania Hospital
Helpful Reviews
Similar Doctors
|
Dr. Marta Rozans, MD
 This Doctor is OpenDoctor Recommended.
|
|
|
Dr. Marie Stuart, MD
This Doctor is OpenDoctor Recommended.
|
|
|
Dr. David T. Teachey, MD
This Doctor is OpenDoctor Recommended.
|
|
|
Dr. Dana M. Sepe, MD
This Doctor is OpenDoctor Recommended.
|
|
|
Dr. Marcia Arem, MD
This Doctor is OpenDoctor Recommended.
|
|
|
Dr. Rebecca Citrin, MD
This Doctor is OpenDoctor Recommended.
|