نتایج جستجو برای: fetal hemoglobin hbf

تعداد نتایج: 142698  

2015
Åsa Nääv Lena Erlandsson Josefin Axelsson Irene Larsson Martin Johansson Lena Wester-Rosenlöf Matthias Mörgelin Vera Casslén Magnus Gram Bo Åkerström Stefan R. Hansson

Preeclampsia is one of the most serious pregnancy-related diseases and clinically manifests as hypertension and proteinuria after 20 gestational weeks. The worldwide prevalence is 3-8% of pregnancies, making it the most common cause of maternal and fetal morbidity and mortality. Preeclampsia lacks an effective therapy, and the only "cure" is delivery. We have previously shown that increased syn...

Journal: :Blood 1998
H L Shear L Grinberg J Gilman M E Fabry G Stamatoyannopoulos D E Goldberg R L Nagel

Studies in vitro by Pasvol et al (Nature, 270:171, 1977) have indicated that the growth of Plasmodium falciparum in cells containing fetal hemoglobin (HbF = alpha2gamma2) is retarded, but invasion is increased, at least in newborn cells. Normal neonates switch from about 80% HbF at birth to a few percent at the end of the first year of life. Carriers of beta-thalassemia trait exhibit a delay in...

2004
Natarajan V. Bhanu Tiffany A. Trice Y. Terry Lee Nicole M. Gantt Patricia Oneal Joseph D. Schwartz Pierre Noel Jeffery L. Miller

We systematically compared cytokinemediated increasesordecreases inproliferation with globin gene and protein expression in adult human erythroblasts. Despite their opposite effects on growth, stem cell factor (SCF) and transforming growth factorbeta (TGF-B) had synergistic effects with respect to fetal hemoglobin (HbF): average HbF/HbF adult hemoglobin (HbA) ratio in erythropoietin (EPO) 1.4 1...

Journal: :Blood 2016
Hetty H Helsmoortel Silvia Bresolin Tim Lammens Hélène Cavé Peter Noellke Aurélie Caye Farzaneh Ghazavi Andrica de Vries Henrik Hasle Veerle Labarque Riccardo Masetti Jan Stary Marry M van den Heuvel-Eibrink Jan Philippé Nadine Van Roy Yves Benoit Frank Speleman Charlotte Niemeyer Christian Flotho Giuseppe Basso Geertruy Te Kronnie Pieter Van Vlierberghe Barbara De Moerloose

Juvenile myelomonocytic leukemia (JMML) is a rare and aggressive stem cell disease of early childhood. RAS activation constitutes the core component of oncogenic signaling. In addition, leukemic blasts in one-fourth of JMML patients present with monosomy 7, and more than half of patients show elevated age-adjusted fetal hemoglobin (HbF) levels. Hematopoietic stem cell transplantation is the cur...

Journal: :The Journal of clinical investigation 1980
J DeSimone P Heller J Amsel M Usman

When hemolytic anemia was induced in 26 baboons (Papio cynocephalus), aged 7-22 mo, they increased their production of fetal hemoglobin (HbF). Although the resulting reduction in hematocrits and increases of reticulocyte counts were similar in all stressed animals there was marked variability in the maximal rates of HbF synthesis. The maximal levels of HbF attained appeared to fall into three s...

Journal: :Blood 1980
J E Barker J E Pierce A W Nienhuis

Stimulation of sheep erythropoietic progenitor cells by erythropoietin (epo) has been studied with regard to its effect on the pattern of hemoglobin production. An analysis of hemoglobin (Hb) synthesis in BFU-E- and CFU-E-derived colonies from fetuses either homozygous for HbA (AA) (homozygous also for the beta c gene responsible for HbC production) or HbB (BB) (lacking the beta c gene) indicat...

2017
Robert Molokie Donald Lavelle Michel Gowhari Michael Pacini Lani Krauz Johara Hassan Vinzon Ibanez Maria A Ruiz Kwok Peng Ng Philip Woost Tomas Radivoyevitch Daisy Pacelli Sherry Fada Matthew Rump Matthew Hsieh John F Tisdale James Jacobberger Mitch Phelps James Douglas Engel Santhosh Saraf Lewis L Hsu Victor Gordeuk Joseph DeSimone Yogen Saunthararajah

BACKGROUND Sickle cell disease (SCD), a congenital hemolytic anemia that exacts terrible global morbidity and mortality, is driven by polymerization of mutated sickle hemoglobin (HbS) in red blood cells (RBCs). Fetal hemoglobin (HbF) interferes with this polymerization, but HbF is epigenetically silenced from infancy onward by DNA methyltransferase 1 (DNMT1). METHODS AND FINDINGS To pharmacol...

Journal: :Blood 2009
Ajay Perumbeti Tomoyasu Higashimoto Fabrizia Urbinati Robert Franco Herbert J Meiselman David Witte Punam Malik

We show that lentiviral delivery of human gamma-globin gene under beta-globin regulatory control elements in hematopoietic stem cells (HSCs) results in sufficient postnatal fetal hemoglobin (HbF) expression to correct sickle cell anemia (SCA) in the Berkeley "humanized" sickle mouse. Upon de-escalating the amount of transduced HSCs in transplant recipients, using reduced-intensity conditioning ...

Journal: :Blood 2000
M Koshy L Dorn L Bressler R Molokie D Lavelle N Talischy R Hoffman W van Overveld J DeSimone

Augmentation of the fetal hemoglobin (HbF) levels is of therapeutic benefit in patients with sickle cell anemia. Hydroxyurea (HU), by increasing HbF, lowers rates of pain crisis, episodes of acute chest syndrome, and requirements for blood transfusions. For patients with no HbF elevation after HU treatment, augmentation of HbF levels by 5-aza-2'-deoxycytidine (5-aza-CdR, decitabine) could serve...

Journal: :Blood 2015
Shuaiying Cui Kim-Chew Lim Lihong Shi Mary Lee Natee Jearawiriyapaisarn Greggory Myers Andrew Campbell David Harro Shigeki Iwase Raymond C Trievel Angela Rivers Joseph DeSimone Donald Lavelle Yogen Saunthararajah James Douglas Engel

Inhibition of lysine-specific demethylase 1 (LSD1) has been shown to induce fetal hemoglobin (HbF) levels in cultured human erythroid cells in vitro. Here we report the in vivo effects of LSD1 inactivation by a selective and more potent inhibitor, RN-1, in a sickle cell disease (SCD) mouse model. Compared with untreated animals, RN-1 administration leads to induced HbF synthesis and to increase...

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