Red blood cell disorders: pathophysiology and new therapeutic approaches

• Grant number: 14/00984-3
• Support Opportunities: Research Projects - Thematic Grants
• Start date: December 01, 2014
• End date: March 31, 2021
• Field of knowledge: Health Sciences - Medicine - Medical Clinics
• Principal Investigator: Fernando Ferreira Costa
• Grantee: Fernando Ferreira Costa
• Host Institution: Centro de Hematologia e Hemoterapia (HEMOCENTRO). Universidade Estadual de Campinas (UNICAMP). Campinas , SP, Brazil
• Pesquisadores principais: Carla Fernanda Franco Penteado ; Margareth Castro Ozelo ; Maria de Fatima Sonati ; Mônica Barbosa de Melo ; Nicola Amanda Conran Zorzetto
• Associated researchers: Aderson da Silva Araujo ; Berengere Koehl ; Bryan Eric Strauss ; Camila Bononi de Almeida ; Carolina Lanaro ; Caroline Le Van Kim ; Chung Man Chin ; Clarisse Lopes de Castro Lobo ; Daniela Maria Ribeiro ; Dulcinéia Martins de Albuquerque ; Erich Vinicius de Paula ; Flávia Costa Leonardo ; Iscia Teresinha Lopes Cendes ; Jean Leandro dos Santos ; Kleber Yotsumoto Fertrin ; Lilian Maria de Castilho ; Magnun Nueldo Nunes dos Santos ; Marcos André Cavalcanti Bezerra ; Marcus Alexandre Finzi Corat ; Marilda de Souza Goncalves ; Mário Angelo Claudino ; Munir Salomao Skaf ; Susan Elisabeth Domingues Costa Jorge ; Tereza Maria Dantas de Medeiros ; Wassim El Nemer
• Associated research grant(s): 16/08342-6 - Multi-user equipment approved in grant 014/00984-3: ECIS (electric cell-substrate impedance sensing), AP.EMU; 15/05062-0 - Multi-User Equipment approved in grant 014/00984-3: Imagestream X MarkII, AP.EMU
• Associated scholarship(s): 19/20560-7 - Evaluation of inflammatory mediators, eosinophils interaction and the effect of tyrosine kinase inhibitors in mast cells of patients with sickle cell anemia, BP.TT; 19/20844-5 - Structural and functional study of human hemoglobin variants, BP.TT; 19/19648-7 - Red blood cells diseases: physiopathology and new therapeutic approaches, BP.TT; + associated scholarships 19/14453-3 - Analysis of the number of f cells in peripheral blood of patients with sickle cell anemia and its correlation with clinical and laboratory parameters, BP.IC; 19/13839-5 - Evaluation of the mechanisms involved in static and flow cell interactions of blood outgrowth endothelial cells (BOECs) from patients with negative BCR-ABL1 myeloproliferative neoplasms, BP.IC; 18/23484-7 - Evaluation of the efficacy and safety of a strategy based on gene therapy with a viral vector for expression of hemopexin in animal models of Sickle Cell Disease, BP.DR; 18/19846-0 - Overexpression of PIPKIIa enzyme gene in normal erythroid cell cultures and patients with hemoglobin H disease, BP.TT; 17/23305-2 - Association between hemolysis intensity and clinical evolution in patients with sickle cell disease, BP.PD; 18/09576-6 - Analysis of the number of f cells in peripheral blood of patients with sickle cell anemia and its correlation with clinical and laboratory parameters, BP.IC; 18/01367-9 - Molecular mechanisms of fetal hemoglobin induction in eritroblasts of patients with bO/bO Thalassemia and in heterozygous individuals with non-deletional hereditary persistence of fetal hemoglobin (Brazilian type), BP.DR; 18/04581-1 - Genotyping and mating programmed for genetically modified animal production with sickle cell anemia and persistence of fetal hemoglobin after birth, BP.IC; 18/03461-2 - Overexpression of PIPKIIa enzyme gene in normal erythroid cell cultures and patients with hemoglobin h disease, BP.TT; 17/21892-8 - Functional analysis of LXN gene in vitro and in vivo and genes global expression evaluation in primary erythroid culture after HbF induction through the compound decitabine, BP.PD; 17/19103-5 - Red blood cell disorders: pathophysiology and new therapeutic approaches, BP.TT; 17/14594-0 - TGF-beta action on leukocyte polarization in sickle cell anemia, BP.PD; 17/11601-6 - Aspects of angiogenesis in Sickle Cell Anemia: the role of platelets, BP.DD; 17/03954-6 - Evaluation of blood pressure and changes in the renin-angiotensin system in an animal model of sickle cell anemia, BP.MS; 16/13224-2 - MicroRNAs expression profile and their influence on asthma experimental model in sickle cell transgenic mice, BP.MS; 16/22061-0 - Genomic editing with CRISPR/Cas9 for production of hereditary persistance of fetal haemoglobin Brazilian type as sickle cell disease treatment strategy, BP.PD; 15/08330-5 - Transcriptomics in placentas of women carrier of sickle cell disease., BP.DR; 15/26929-1 - In vivo evaluation of the role of eosinophil in the vaso-occlusion process using sickle cell transgenic mice: Involvement of Rho-quinase pathway, BP.MS; 16/08729-8 - Investigation of the inflammasome formation in patients with sickle cell anemia, BP.DD; 16/08072-9 - Characterization of molecular changes in patients with aceruloplasminemia, BP.MS; 15/18369-6 - Evaluation of the role of platelets in sickle cell disease, BP.DR; 16/05014-8 - Evaluation of static and flow cell interactions of blood outgrowth endothelial cells mechanisms in patients with Polycythemia Vera, BP.IC; 16/04706-3 - Standardization of human hemoglobin purification and crystallization, BP.IC; 15/24666-3 - Investigation of new pathophysiological mechanisms of hypercoagulability in Sickle Cell Disease, BP.DR; 15/21184-8 - THP-1 culturing cells standardization to evaluate miRNAs and their target genes response against influence of anti-inflammatory stimuli, BP.IC; 15/24029-3 - Analysis of susceptibility genes to leg ulcer in patients with sickle cell disease, BP.DD; 15/22192-4 - Evaluation of adhesive, procoagulant and inflammatory properties mediated by in vitro interactions of circulating progenitor endothelial cells and endothelial cells differentiated from IPS of patients with sickle cell disease, BP.PD; 15/16188-4 - Hybrids of thalidomide + furoxan derivatives: evaluation of anti-inflammatory properties and induction of gamma globin in vitro, BP.MS; 15/16517-8 - Inflammatory mediators, interaction with eosinophils and tyrosine kinase inhibitors effects on mast cells of sickle cell anemia patients, BP.PD; 15/13970-3 - Functional analysis of LXN gene in vitro and in vivo and genes global expression evaluation in primary erythroid culture after HbF induction through the compound decitabine, BP.PD; 15/13710-1 - Structural and functional study of human hemoglobin variants, BP.PD; 15/14255-6 - RNA-seq technique application in endothelial and leukocyte cells transcription profile evaluation in sickle cell retinopathy, BP.PD; 15/07637-0 - Morphological characteristics of leukocytes and platelets from patients with sickle cell anemia and thalassemia: analysis by image cytometry (Amnis ImageStream), BP.PD; 15/04485-4 - Investigation of the renin-angiotensin system in mice with sickle cell Anemia, BP.IC; 15/04179-0 - Western Blotting technique standardization of protein quantification after PIPKIIa enzyme gene overexpression in human erythroid cell cultures, BP.IC - associated scholarships

Abstract

This new Multi-PI thematic project proposal will be conducted by the Red Blood Cell Research Group, which is formed by researchers from the Hemocentro (Blood Center), the Department of Clinical Pathology, School of Medical Sciences and the Molecular Biology and Genetic Engineering Center (CBMEG) at UNICAMP, in collaboration with several Brazilian and international researchers. The new project represents a major expansion of the previous proposal with the inclusion of new and unprecedented themes, which will undoubtedly result in extremely significant data, from a scientific point of view, and improvements in the therapy of the diseases studied. This project aims to reveal important aspects of red blood cell disease pathophysiology and to discover new forms of treatment, using state of the art equipment and technology, and to broaden the international collaborations formed in recent years. New methods and equipment will be introduced; an intravital microscopy platform for investigation of the microcirculation in vivo, the Amnis ImageStream equipment for the visualization of interactions and cellular alterations, studies using iPS techniques and ultimate generation gene sequencing and functional methods. Taken as a whole, the project proposes studies in five broad areas related to changes in erythrocytes and subsequent diseases; the identification of genes associated with erythropoiesis and molecular factors that regulate the production of HbF, the effects of hemolysis and identification of therapeutic targets, structural and functional changes of the hemoglobin molecule, aspects of the pathophysiology of red blood cell-related diseases and, finally, new drugs and therapeutic targets for the Hemoglobinopathies. The exceptional team of Brazilian and foreign researchers involved in the proposal, associated with the use of methods and equipment described, represents a modern multidisciplinary approach with the use of in vitro and in vivo methods for researching aspects of red cell diseases that have not yet been clarified. We believe that this approach will provide fundamental data for the knowledge of these diseases and the development of new therapies for their treatment. (AU)

Scientific publications

(66)

(References retrieved automatically from Web of Science and SciELO through information on FAPESP grants and their corresponding numbers as mentioned in the publications by the authors)

PEDROSO, GISELE A.; KIMURA, ELZA M.; SANTOS, MAGNUN N. N.; ALBUQUERQUE, DULCINEIA M.; MALIMPENSA, DANAE; JORGE, SUSAN E.; VERISSIMO, MONICA P. A.; COSTA, FERNANDO F.; SONATI, MARIA F.. Thalassemia major phenotype caused by HB Zurich-Albisrieden [alpha 2 59(E8) Gly > Arg (HBA2:C.178G > C)] in a Brazilian child. PEDIATRIC BLOOD & CANCER, v. 65, n. 12, DEC 2018. (14/00984-3, 15/13710-1)

CRUZ, PEDRO R. S.; ANANINA, GALINA; GIL-DA-SILVA-LOPES, VERA LUCIA; SIMIONI, MILENA; MENAA, FARID; BEZERRA, MARCOS A. C.; DOMINGOS, IGOR F.; ARAUJO, ADERSON S.; PELLEGRINO, RENATA; HAKONARSON, HAKON; et al. Genetic comparison of sickle cell anaemia cohorts from Brazil and the United States reveals high levels of divergence. SCIENTIFIC REPORTS, v. 9, JUL 26 2019. (08/10596-0, 08/57441-0, 14/00984-3, 12/06438-5, 15/13152-9)

MARIA SOLER, ANA; SCHELOTTO, MAGDALENA; DE OLIVEIRA MOTA, NATALIA; DORTA FERREIRA, ROBERTA; DE FATIMA SONATI, MARIA; ABAYUBA DA LUZ, JULIO. The -()(5.2) Deletion Detected in a Uruguayan Family: First Case Report in the Americas. HEMOGLOBIN, v. 40, n. 4, p. 289-292, AUG 2016. (14/00984-3)

TORRES, LIDIANE; CONRAN, NICOLA. Emerging pharmacotherapeutic approaches for the management of sickle cell disease. EXPERT OPINION ON PHARMACOTHERAPY, v. 20, n. 2, p. 173-186, JAN 22 2019. (17/14594-0, 14/00984-3)

JORGE, S. E.; BRINGAS, M.; PETRUK, A. A.; ARRAR, M.; MARTI, M. A.; SKAF, M. S.; COSTA, F. F.; CAPECE, L.; SONATI, M. F.; ESTRIN, D.. Understanding the molecular basis of the high oxygen affinity variant human hemoglobin Coimbra. Archives of Biochemistry and Biophysics, v. 637, p. 73-78, JAN 1 2018. (14/00984-3, 15/13710-1)

SILVA, FABIO H.; CLAUDINO, MARIO A.; CALMASINI, FABIANO B.; ALEXANDRE, EDUARDO C.; FRANCO-PENTEADO, CARLA; BURNETT, ARTHUR L.; ANTUNES, EDSON; COSTA, FERNANDO F.. Sympathetic Hyperactivity, Increased Tyrosine Hydroxylase and Exaggerated Corpus Cavernosum Relaxations Associated with Oxidative Stress Plays a Major Role in the Penis Dysfunction in Townes Sickle Cell Mouse. PLoS One, v. 11, n. 12, DEC 9 2016. (14/00984-3, 13/19781-2)

SILVA, FABIO H.; KARAKUS, SERKAN; MUSICKI, BILJANA; MATSUI, HOTAKA; BIVALACQUA, TRINITY J.; DOS SANTOS, JEAN L.; COSTA, FERNANDO F.; BURNETT, ARTHUR L.. Beneficial Effect of the Nitric Oxide Donor Compound 3-(1,3-Dioxoisoindolin-2-yl)Benzyl Nitrate on Dysregulated Phosphodiesterase 5, NADPH Oxidase, and Nitrosative Stress in the Sickle Cell Mouse Penis: Implication for Priapism Treatment. Journal of Pharmacology and Experimental Therapeutics, v. 359, n. 2, p. 230-237, NOV 1 2016. (14/00984-3, 10/12495-6, 13/19781-2, 14/21965-7)

BAPTISTA, LETICIA C.; COSTA, MARIA LAURA; FERREIRA, REGIANE; ALBUQUERQUE, DULCINEIA M.; LANARO, CAROLINA; FERTRIN, KLEBER Y.; SURITA, FERNANDA G.; PARPINELLI, MARY A.; COSTA, FERNANDO F.; DE MELO, MONICA BARBOSA. Abnormal expression of inflammatory genes in placentas of women with sickle cell anemia and sickle hemoglobin C disease. ANNALS OF HEMATOLOGY, v. 95, n. 11, p. 1859-1867, NOV 2016. (14/00984-3, 08/57441-0)

LIMA, KELI; RIBEIRO, DANIELA MARIA; CAMPOS, PAULA DE MELO; COSTA, FERNANDO FERREIRA; TRAINA, FABIOLA; OLALLA SAAD, SARA TERESINHA; SONATI, MARIA DE FATIMA; MACHADO-NETO, JOAO AGOSTINHO. Differential profile of PIP4K2A expression in hematological malignancies. BLOOD CELLS MOLECULES AND DISEASES, v. 55, n. 3, p. 228-235, OCT 2015. (11/51959-0, 14/00984-3)

LEONARDO, DANIELA P.; ALBUQUERQUE, DULCINELA M.; LANARO, CAROLINA; BAPTISTA, LETICIA C.; CECATTI, JOSE G.; SURITA, FERNANDA G.; PARPINELLI, MARY A.; COSTA, FERNANDO F.; FRANCO-PENTEADO, CARLA F.; FERTRIN, KLEBER Y.; et al. Association of Nitric Oxide Synthase and Matrix Metalloprotease Single Nucleotide Polymorphisms with Preeclampsia and Its Complications. PLoS One, v. 10, n. 8, AUG 28 2015. (14/00984-3, 08/57441-0)

MOTA, NATALIA O.; KIMURA, ELZA M.; FERREIRA, ROBERTA D.; PEDROSO, GISELE A.; ALBUQUERQUE, DULCINEIA M.; RIBEIRO, DANIELA M.; SANTOS, MAGNUN N. N.; BITTAR, CRISTINA M.; COSTA, FERNANDO F.; SONATI, MARIA DE FATIMA. Rare alpha(0)-thalassemia deletions detected by MLPA in five unrelated Brazilian patients. GENETICS AND MOLECULAR BIOLOGY, v. 40, n. 4, p. 768-773, OCT-DEC 2017. (14/00984-3, 15/21184-8)

SILVA, FABIO HENRIQUE; FERTRIN, KLEBER YOTSUMOTO; ALEXANDRE, EDUARDO COSTA; CALMASINI, FABIANO BERALDI; FRANCO-PENTEADO, CARLA FERNANDA; COSTA, FERNANDO FERREIRA. Impairment of Nitric Oxide Pathway by Intravascular Hemolysis Plays a Major Role in Mice Esophageal Hypercontractility: Reversion by Soluble Guanylyl Cyclase Stimulator. Journal of Pharmacology and Experimental Therapeutics, v. 367, n. 2, p. 194-202, NOV 1 2018. (14/00984-3, 18/06243-6, 17/08122-9)

ITO, MIRTA T.; DA SILVA COSTA, SUELI M.; BAPTISTA, LETICIA C.; CARVALHO-SIQUEIRA, GABRIELA Q.; ALBUQUERQUE, DULCINEIA M.; RIOS, VINICIUS M.; OSPINA-PRIETO, STEPHANIE; SAEZ, ROBERTA C.; VIEIRA, KARLA P.; CENDES, FERNANDO; et al. Angiogenesis-Related Genes in Endothelial Progenitor Cells May Be Involved in Sickle Cell Stroke. JOURNAL OF THE AMERICAN HEART ASSOCIATION, v. 9, n. 3, FEB 4 2020. (14/00984-3)

CHINEDU, OKEKE; TONASSE, WOUITCHEKPO VINCENT; ALBUQUERQUE, DULCINEIA MARTINS; DOMINGOS, IGOR DE FARIAS; ARAUJO, ADERSON DA SILVA; CAVALCANTI BEZERRA, MARCOS ANDRE; SONATI, MARIA DE FATIMA; NUNES DOS SANTOS, MAGNUN NUELDO. Polymorphisms in the heme oxygenase-1 and bone morphogenetic protein receptor type 1b genes and estimated glomerular filtration rate in Brazilian sickle cell anemia patients. Hematology, Transfusion and Cell Therapy, v. 43, n. 2, p. 165-170, APR-JUN 2021. (14/00984-3)

PEDROSO, G. A.; FERNANDES, P.; JORGE, S. E. D. C.; NASCIMENTO, P. H.; LIMA, P. C.; GRIGOLETO, M. R. P.; ALBUQUERQUE, D. M.; SANTOS, M. N. N.; COSTA, F. F.; TORO, A. A. D. C.; et al. Hemoglobin Kirklareli [Alpha(2) 59(E7) His >>Leu; HBA2:c.176A > T] in a Brazilian child with severe dyspnea and low O-2 saturation. ANNALS OF HEMATOLOGY, v. 98, n. 12, p. 2853-2855, DEC 2019. (15/13710-1, 14/00984-3)

SANTOS, T. D.; MACEDO, M. D.; MENEGATI, S. F. P.; GILLI, S.; CASTILHO, L.. Challenges in providing compatible blood with Rh genotype-matching in Brazilian patients with sickle cell disease. Transfusion Medicine, OCT 2019. (14/00984-3, 15/07559-9)

CHENOU, FRANCINE; HOUNKPE, BIDOSSESSI WILFRIED; DOMINGOS, IGOR DE FARIAS; TONASSE, WOUITCHEKPO VINCENT; CHAVES BATISTA, THAIS HELENA; SANTANA, RODRIGO MARCIONILO; ARCANJO, GABRIELA DA SILVA; ALAGBE, ADEKUNLE EMMANUEL; ARAUJO, ADERSON DA SILVA; LUCENA-ARAUJO, ANTONIO ROBERTO; et al. Effect of hydroxyurea therapy on intravascular hemolysis and endothelial dysfunction markers in sickle cell anemia patients. ANNALS OF HEMATOLOGY, v. 100, n. 11, AUG 2021. (14/00984-3)

OLATUNYA, OLADELE SIMEON; LANARO, CAROLINA; LONGHINI, ANA LEDA; FRANCO PENTEADO, CARLA FERNANDA; FERTRIN, KLEBER Y.; ADEKILE, ADEKUNLE; SAAD, SARA T. O.; COSTA, FERNANDO FERREIRA. Red blood cells microparticles are associated with hemolysis markers and may contribute to clinical events among sickle cell disease patients. ANNALS OF HEMATOLOGY, v. 98, n. 11, p. 2507-2521, NOV 2019. (14/00984-3)

TORRES, LIDIANE S.; CHWEIH, HANAN; FABRIS, FERNANDA C. Z.; GOTARDO, ERICA M. F.; LEONARDO, FLAVIA C.; OLALLA SAAD, SARA T.; COSTA, FERNANDO F.; CONRAN, NICOLA. TGF-beta 1 Reduces Neutrophil Adhesion and Prevents Acute Vaso-Occlusive Processes in Sickle Cell Disease Mice. CELLS, v. 11, n. 7, p. 12-pg., 2022-04-01. (19/18886-1, 14/00984-3, 17/14594-0)

TOLEDO DEL RIO, ANA PAULA; FRADE-GUANAES, JESSICA O.; OSPINA-PRIETO, STEPHANIE; DUARTE, BRUNO K. L.; BERTOLO, MANOEL BARROS; OZELO, MARGARETH C.; SACHETTO, ZORAIDA. Impaired repair properties of endothelial colony-forming cells in patients with granulomatosis with polyangiitis. JOURNAL OF CELLULAR AND MOLECULAR MEDICINE, v. 26, n. 19, p. 10-pg., 2022-09-02. (14/00984-3)

CONRAN, NICOLA; DE ALVARENGA MAXIMO, CLAUDIA; OLIVEIRA, THAIS; FERTRIN, KLEBER Y.; LOBO, CLARISSE; COSTA, FERNANDO F.. Safe use of hydroxycarbamide in sickle cell disease patients hospitalized for painful vaso-occlusive episodes during the randomized, open-label HELPS study. British Journal of Haematology, v. 199, n. 1, p. 5-pg., 2022-07-14. (14/00984-3)

OLATUNYA, OLADELE S.; ALBUQUERQUE, DULCINEIA M.; ADEKILE, ADEKUNLE; COSTA, FERNANDO F.. Influence of alpha thalassemia on clinical and laboratory parameters among nigerian children with sickle cell anemia. JOURNAL OF CLINICAL LABORATORY ANALYSIS, v. 33, n. 2, FEB 2019. (14/00984-3)

FERREIRA DE MELO, THAIS REGINA; KUMKHAEK, CHUTIMA; DOS SANTOS FERNANDES, GUILHERME FELIPE; LOPES PIRES, MARIA ELISA; CHELUCCI, RAFAEL CONSOLIN; BARBIERI, KARINA PEREIRA; COELHO, FERNANDA; DE OLIVEIRA CAPOTE, TICIANA SIDORENKO; LANARO, CAROLINA; CARLOS, IRACILDA ZEPPONE; et al. Discovery of phenylsulfonylfuroxan derivatives as gamma globin inducers by histone acetylation. EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY, v. 154, p. 341-353, JUN 25 2018. (10/12495-6, 14/00984-3, 16/09502-7, 13/04244-1, 14/06755-6)

MAGNUN NUELDO NUNES DOS SANTOS. Haptoglobin: an emerging candidate for phenotypic modulation of sickle cell anemia?. Revista Brasileira de Hematologia e Hemoterapia, v. 37, n. 6, p. 361-363, 2015-12-00. (14/00984-3)

BOSQUESI, PRISCILA LONGHIN; BOLOGNESI MELCHIOR, AYLIME CASTANHO; PAVAN, ALINE RENATA; LANARO, CAROLINA; DE SOUZA, CRISTIANE MARIA; RUSINOVA, RADDA; CHELUCCI, RAFAEL CONSOLIN; BARBIERI, KARINA PEREIRA; DOS SANTOS FERNANDES, GUILHERME FELIPE; CARLOS, IRACILDA ZEPONE; et al. Synthesis and evaluation of resveratrol derivatives as fetal hemoglobin inducers. BIOORGANIC CHEMISTRY, v. 100, JUL 2020. (14/00984-3, 12/50359-2)

PIMENTA SANTOS, MARINA ERE HUMMEL; OLOPS, LETICIA; VENDRAME, FELIPE; JUNQUEIRA TAVARES, ALVARO HENRIQUE; LEONARDO, DANIELA PINHEIRO; DE AZEVEDO, PAULA CHRISTINA; PIOVESANA, LUIZA GONZAGA; COSTA, FERNANDO FERREIRA; FERTRIN, KLEBER YOTSUMOTO. Benserazide as a potential novel fetal hemoglobin inducer: an observational study in non-carriers of hemoglobin disorders. BLOOD CELLS MOLECULES AND DISEASES, v. 87, MAR 2021. (14/00984-3)

SESTI-COSTA, RENATA; BORGES, MARINA DORIGATTI; LANARO, CAROLINA; DE ALBUQUERQUE, DULCINEIA MARTINS; OLALLA SAAD, SARA TEREZINHA; COSTA, FERNANDO FERREIRA. Inflammatory Dendritic Cells Contribute to Regulate the Immune Response in Sickle Cell Disease. FRONTIERS IN IMMUNOLOGY, v. 11, FEB 4 2021. (14/00984-3, 17/21892-8)

HOUNKPE, BIDOSSESSI WILFRIED; CHENOU, FRANCINE; DOMINGOS, IGOR DE FARIAS; CARDOSO, EVILAZIO CUNHA; COSTA SOBREIRA, MARCONDES JOSE DE VASCONCELOS; ARAUJO, ADERSON S.; LUCENA-ARAUJO, ANTONIO ROBERTO; DA SILVA NETO, PEDRO VIEIRA; MALHEIRO, ADRIANA; FRAIJI, NELSON ABRAHIM; et al. Neutrophil extracellular trap regulators in sickle cell disease: Modulation of gene expression of PADI4, neutrophil elastase, and myeloperoxidase during vaso-occlusive crisis. RESEARCH AND PRACTICE IN THROMBOSIS AND HAEMOSTASIS, v. 5, n. 1, DEC 2020. (14/00984-3, 15/24666-3, 16/14172-6)

GOMES SANTATERRA, VANESSA ARAUJO; LUZ FIUSA, MAIARA MARX; HOUNKPE, BIDOSSESSI WILFRIED; CHENOU, FRANCINE; TONASSE, WOUITCHEKPO VINCENT; GOMES DA COSTA, LOREDANA NILKENES; GARCIA-WEBER, DIEGO; DOMINGOS, IGOR DE FARIAS; DE LIMA, FRANCIELE; BORBA-JUNIOR, IVANIO TEIXEIRA; et al. Endothelial Barrier Integrity Is Disrupted In Vitro by Heme and by Serum From Sickle Cell Disease Patients. FRONTIERS IN IMMUNOLOGY, v. 11, DEC 14 2020. (14/00984-3, 16/14172-6)

CHENOU, F.; ALBUQUERQUE, D. M.; LEONARDO, D. P.; DOMINGOS, I. F.; BEZERRA, M. A. C.; ARAUJO, A. S.; BLOTTA, M. H. S. L.; COSTA, F. F.; SONATI, M. F.; PAULA, V, E.; et al. Endothelial Nitric Oxide Synthase (eNOS) Gene Polymorphisms and Markers of Hemolysis, Inflammation and Endothelial Dysfunction in Brazilian Sickle Cell Anemia Patients. BIOCHEMICAL GENETICS, v. 58, n. 4, p. 580-594, AUG 2020. (14/00984-3)

SOUSA DA CRUZ, PEDRO RODRIGUES; ANANINA, GALINA; SECOLIN, RODRIGO; GIL-DA-SILVA-LOPES, VERA LUCIA; PASSOS LIMA, CARMEN SILVIA; CONDEIXA DE FRANCA, PAULO HENRIQUE; DONATTI, AMANDA; LOURENCO, GUSTAVO JACOB; DE ARAUJO, TANIA KAWASAKI; SIMIONI, MILENA; et al. Demographic history differences between Hispanics and Brazilians imprint haplotype features. G3-GENES, GENOMES, GENETICS, v. N/A, p. 13-pg., 2022-05-02. (08/57441-0, 13/07559-3, 14/00984-3, 15/13152-9, 19/18886-1, 08/10596-0, 12/06438-5)

MACEDO, MAYRA D.; MIRANDA, MARIA R.; SANTOS, TAMIRES D.; LEAL, IANCA; CASTILHO, LILIAN. Rh antibodies as a result of altered Rh epitopes on transfused red cells: a case series of seven Brazilian patients. BLOOD TRANSFUSION, v. 19, n. 5, p. 413-419, SEP-OCT 2021. (14/00984-3, 15/07559-9)

GIL, GISLENE PEREIRA; ANANINA, GALINA; MASCHIETTO, MARIANA; LIMA, SHEILA COELHO SOARES; DA SILVA COSTA, SUELI MATILDE; BAPTISTA, LETICIA DE CARVALHO; ITO, MIRTA TOMIE; COSTA, FERNANDO FERREIRA; COSTA, MARIA LAURA; DE MELO, MONICA BARBOSA. Epigenetic analysis in placentas from sickle cell disease patients reveals a hypermethylation profile. PLoS One, v. 17, n. 9, p. 23-pg., 2022-09-21. (14/00984-3, 19/18886-1)

DA SILVA COSTA, SUELI MATILDE; ITO, MIRTA; SOUZA, BRUNO BATISTA; SOUZA CRUZ, PEDRO RODRIGUES; MONTEIRO VITURINO, MARINA GONCALVES; PRIETO, STEPHANIE OSPINA; SAEZ, ROBERTA CASAGRANDE; OZELO, MARGARETH CASTRO; COSTA, FERNANDO FERREIRA; MELO, MONICA B.. Identification of candidate genes involved in proliferative sickle cell retinopathy by RNAseq.. INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE, v. 59, n. 9, p. 3-pg., 2018-07-01. (14/00984-3, 15/14255-6)

NATÁLIA O. MOTA; ELZA M. KIMURA; ROBERTA D. FERREIRA; GISELE A. PEDROSO; DULCINÉIA M. ALBUQUERQUE; DANIELA M. RIBEIRO; MAGNUN N. N. SANTOS; CRISTINA M. BITTAR; FERNANDO F. COSTA; MARIA DE FATIMA SONATI. Rare α0-thalassemia deletions detected by MLPA in five unrelated Brazilian patients. GENETICS AND MOLECULAR BIOLOGY, v. 40, n. 4, p. 768-773, 2017-10-02. (15/21184-8, 14/00984-3)

OLATUNYA, OLADELE SIMEON; ALBUQUERQUE, DULCINEIA MARTINS; AKANBI, GANIYU OLUSOLA; ADUAYI, OLUFUNSO SIMISOLA; TAIWO, ADEKUNLE BAMIDELE; FABOYA, OPEYEMI AYODEJI; KAYODE, TOLORUNJU SEGUN; LEONARDO, DANIELA PINHEIRO; ADEKILE, ADEKUNLE; COSTA, FERNANDO FERREIRA. Uridine diphosphate glucuronosyl transferase 1A (UGT1A1) promoter polymorphism in young patients with sickle cell anaemia: report of the first cohort study from Nigeria. BMC MEDICAL GENETICS, v. 20, n. 1, OCT 16 2019. (14/00984-3)

CONRAN, NICOLA; DE PAULA, ERICH V.. Thromboinflammatory mechanisms in sickle cell disease - challenging the hemostatic balance. Haematologica, v. 105, n. 10, p. 2380-2390, OCT 2020. (14/00984-3)

MENEGATI, SHEILA F. P.; SANTOS, TAMIRES D.; MACEDO, MAYRA D.; CASTILHO, LILIAN. Discrepancies between red cell phenotyping and genotyping in daily immunohematology laboratory practice. TRANSFUSION AND APHERESIS SCIENCE, v. 59, n. 1, FEB 2020. (14/00984-3, 15/07559-9)

DOMINGOS, IGOR F.; PEREIRA-MARTINS, DIEGO A.; SOBREIRA, MARCONDES J. V. C.; OLIVEIRA, ROMULO T. D.; ALAGBE, ADEKUNLE E.; LANARO, CAROLINA; ALBUQUERQUE, DULCINEIA M.; BLOTTA, MARIA H. S. L.; ARAUJO, ADERSON S.; COSTA, FERNANDO F.; et al. High levels of proinflammatory cytokines IL-6 and IL-8 are associated with a poor clinical outcome in sickle cell anemia. ANNALS OF HEMATOLOGY, v. 99, n. 5, p. 947-953, MAY 2020. (14/00984-3)

OLATUNYA, OLADELE SIMEON; ALBUQUERQUE, DULCINEIA MARTINS; SANTOS, MAGNUN NUELDO NUNES; KAYODE, TOLORUNJU SEGUN; ADEKILE, ADEKUNLE; COSTA, FERNANDO FERREIRA. Haptoglobin Gene Polymorphism in Patients with Sickle Cell Anemia: Findings from a Nigerian Cohort Study. APPLICATION OF CLINICAL GENETICS, v. 13, p. 107-114, 2020. (14/00984-3)

JORGE, S. E.; LANARO, C.; ALBUQUERQUE, D. M.; NASCIMENTO, P. H.; PEDROSO, G. A.; OLIVEIRA, S. C.; GRIGOLETO, M. R. P.; SANTOS, M. N. N.; COSTA, F. F.; SONATI, M. F.. Hb Fairfax [HBB:c.285_286insGAGCTGCACTGTGAC] in a Brazilian patient with severe hemolytic anemia-identification and functional study. ANNALS OF HEMATOLOGY, v. 98, n. 9, p. 2253-2255, SEP 2019. (15/13710-1, 14/00984-3)

SANTOS, BRUNA CUNHA; JORGE, SUSAN ELISABETH; DE ALBUQUERQUE, DULCINEIA MARTINS; OLENSCKI GILLI, SIMONE CRISTINA; SONATI, MARIA DE FATIMA; FERTRIN, KLEBER YOTSUMOTO; COSTA, FERNANDO FERREIRA. High erythropoietin may be associated with vascular complications in patients with secondary erythrocytosis caused by high oxygen affinity variant hemoglobin Coimbra. BLOOD CELLS MOLECULES AND DISEASES, v. 79, NOV 2019. (14/00984-3)

BORGES, MARINA DORIGATTI; DE ALBUQUERQUE, DULCINEIA MARTINS; LANARO, CAROLINA; COSTA, FERNANDO FERREIRA; FERTRIN, KLEBER YOTSUMOTO. Clinical relevance of heterozygosis for aceruloplasminemia. AMERICAN JOURNAL OF MEDICAL GENETICS PART B-NEUROPSYCHIATRIC GENETICS, v. 180, n. 4, p. 266-271, JUN 2019. (16/08072-9, 14/00984-3)

SILVA, JULIETE A. F.; GOTARDO, ERICA M. F.; CHWEIH, HANAN; MIGUEL, LEDIANA I.; FERREIRA, JR., WILSON A.; HEDLUND, BO; ELFORD, HOWARD L.; LEONARDO, FLAVIA C.; COSTA, FERNANDO F.; CONRAN, NICOLA. Didox (3,4-dihydroxybenzohydroxamic acid) reduces the vascular inflammation induced by acute intravascular hemolysis. BLOOD CELLS MOLECULES AND DISEASES, v. 81, MAR 2020. (14/00984-3)

VENDRAME, FELIPE; OLOPS, LETICIA; OLALLA SAAD, SARA TERESINHA; FERREIRA COSTA, FERNANDO; YOTSUMOTO FERTRIN, KLEBER. Hypocholesterolemia and dysregulated production of angiopoietin-like proteins in sickle cell anemia patients. CYTOKINE, v. 120, p. 88-91, AUG 2019. (14/00984-3)

CONRAN, NICOLA; TORRES, LIDIANE. cGMP modulation therapeutics for sickle cell disease. Experimental Biology and Medicine, v. 244, n. 2, p. 132-146, FEB 2019. (17/14594-0, 18/08010-9, 14/00984-3)

FERREIRA, R. D.; MOTA, N. O.; PEDROSO, G. A.; KIMURA, E. M.; GERALDO, A. P. M.; SANTOS, M. N. N.; COSTA, F. F.; SONATI, M. F.. First report of epsilon(0)-thalassemia in a Brazilian family. INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, v. 40, n. 5, p. E96-E98, OCT 2018. (14/00984-3)

LERCHNER, J.; VOLPE, P. O. L.; LANARO, C.; FERTRIN, K. Y.; COSTA, F. F.; ALBUQUERQUE, D. M.; HAENSCHKE, F.; MERTENS, F.. A chip calorimetry-based method for the real-time investigation of metabolic activity changes in human erythrocytes caused by cell sickling. JOURNAL OF THERMAL ANALYSIS AND CALORIMETRY, v. 136, n. 2, p. 771-781, APR 2019. (14/00984-3)

BAPTISTA, LETICIA C.; FIGUEIRA, CAMILLA O.; SOUZA, BRUNO B.; FERTRIN, KLEBER Y.; ANTOLINI, ARTHUR; COSTA, FERNANDO F.; DE MELO, MONICA B.; COSTA, MARIA LAURA. Highlight Article: Different morphological and gene expression profile in placentas of the same sickle cell anemia patient in pregnancies of opposite outcomes. Experimental Biology and Medicine, v. 244, n. 5, p. 395-403, APR 2019. (15/08330-5, 14/00984-3)

MIRANDA, MARIA RITA; DOS SANTOS, TAMIRES DELFINO; CASTILHO, LILIAN. Systematic RHD genotyping in Brazilians reveals a high frequency of partial D in transfused patients serologically typed as weak D. TRANSFUSION AND APHERESIS SCIENCE, v. 60, n. 6, DEC 2021. (14/00984-3, 20/02191-1)

CONRAN, NICOLA; EMBURY, STEPHEN H.. Sickle cell vaso-occlusion: The dialectic between red cells and white cells. Experimental Biology and Medicine, v. 246, n. 12, SI, p. 1458-1472, JUN 2021. (14/00984-3, 18/08010-9)

DE CARVALHO-SIQUEIRA, GABRIELA QUEILA; ANANINA, GALINA; DE SOUZA, BRUNO BATISTA; BORGES, MURILO GUIMARAES; ITO, MIRTA TOMIE; DA SILVA-COSTA, SUELI MATILDE; DOMINGOS, IGOR DE FARIAS; FALCAO, DIEGO ARRUDA; LOPES-CENDES, ISCIA; CAVALCANTI BEZERRA, MARCOS ANDRE; et al. Highlight article: Whole-exome sequencing indicates FLG2 variant associated with leg ulcers in Brazilian sickle cell anemia patients. Experimental Biology and Medicine, v. 244, n. 11, p. 932-939, AUG 2019. (15/24029-3, 14/00984-3)

DUARTE, BRUNO K. L.; YAMAGUTI-HAYAKAWA, GABRIELA G.; MEDINA, SAMUEL S.; SIQUEIRA, LUCIA H.; SNETSINGER, BROOKE; COSTA, FERNANDO F.; RAUH, MICHAEL J.; OZELO, MARGARETH C.. Longitudinal sequencing of RUNX1 familial platelet disorder: new insights into genetic mechanisms of transformation to myeloid malignancies. British Journal of Haematology, v. 186, n. 5, p. 724-734, SEP 2019. (14/00984-3)

FERREIRA DE MELO, THAIS REGINA; DULMOVITS, BRIAN M.; DOS SANTOS FERNANDES, GUILHERME FELIPE; DE SOUZA, CRISTIANE M.; LANARO, CAROLINA; HE, MINGHZU; AL ABED, YOUSEF; CHUNG, MAN CHIN; BLANC, LIONEL; COSTA, FERNANDO FERREIRA; et al. Synthesis and pharmacological evaluation of pomalidomide derivatives useful for sickle cell disease treatment. BIOORGANIC CHEMISTRY, v. 114, SEP 2021. (16/09502-7, 15/19531-1, 13/04244-1, 10/12495-6, 14/00984-3, 14/06755-6)

PINHEIRO, ANDRESSA KELY; PEREIRA, DALILA ANDRADE; DOS SANTOS, JEAN LEANDRO; CALMASINI, FABIANO BERALDI; ALEXANDRE, EDUARDO COSTA; REIS, LEONARDO OLIVEIRA; BURNETT, ARTHUR L.; COSTA, FERNANDO FERREIRA; SILVA, FABIO HENRIQUE. Resveratrol-nitric oxide donor hybrid effect on priapism in sickle cell and nitric oxide-deficient mouse. PLoS One, v. 17, n. 6, p. 13-pg., 2022-01-01. (12/50359-2, 14/00984-3)

BAPTISTA, LETICIA CARVALHO; COSTA, MARIA LAURA; SURITA, FERNANDA GARANHANI; ROCHA, CRISTIANE DE SOUZA; LOPES-CENDES, ISCIA; DE SOUZA, BRUNO BATISTA; COSTA, FERNANDO FERREIRA; DE MELO, MONICA BARBOSA. Placental transcriptome profile of women with sickle cell disease reveals differentially expressed genes involved in migration, trophoblast differentiation and inflammation. BLOOD CELLS MOLECULES AND DISEASES, v. 84, SEP 2020. (15/08330-5, 14/00984-3)

VILA CUENCA, MARC; MARCHI, GIACOMO; BARQUE, ANNA; ESTEBAN-JURADO, CLARA; MARCHETTO, ALESSANDRO; GIORGETTI, ALEJANDRO; CHELBAN, VIORICA; HOULDEN, HENRY; WOOD, NICHOLAS W.; PIUBELLI, CHIARA; et al. Genetic and Clinical Heterogeneity in Thirteen New Cases with Aceruloplasminemia. Atypical Anemia as a Clue for an Early Diagnosis. INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES, v. 21, n. 7, APR 2020. (16/08072-9, 14/00984-3)

GASPARDI, ANE C.; SIPPERT, EMILIA A.; DE MACEDO, MAYRA DORIGAN; PELLEGRINO, JR., JORDAO; COSTA, FERNANDO F.; CASTILHO, LILIAN. Clinically relevant RHD-CE genotypes in patients with sickle cell disease and in African Brazilian donors. BLOOD TRANSFUSION, v. 14, n. 5, p. 449-454, 2016. (15/07559-9, 14/00984-3)

VENDRAME, FELIPE; OLOPS, LETICIA; SAAD, SARA TERESINHA OLALLA; COSTA, FERNANDO FERREIRA; FERTRIN, KLEBER YOTSUMOTO. Differences in heme and hemopexin content in lipoproteins from patients with sickle cell disease. JOURNAL OF CLINICAL LIPIDOLOGY, v. 12, n. 6, p. 1532-1538, DEC 2018. (14/00984-3)

DE SOUZA, GLEICE REGINA; HOUNKPE, BIDOSSESSI WILFRIED; LUZ FIUSA, MAIARA MARX; COLELLA, MARINA PEREIRA; ANNICHINO-BIZZACCHI, JOYCE M.; TRAINA, FABIOLA; COSTA, FERNANDO FERREIRA; DE PAULA, ERICH VINICIUS. Tissue factor-dependent coagulation activation by heme: A thromboelastometry study. PLoS One, v. 12, n. 4, APR 24 2017. (15/24666-3, 14/00984-3, 13/09319-0)

. Rare α0-thalassemia deletions detected by MLPA in five unrelated Brazilian patients. GENETICS AND MOLECULAR BIOLOGY, n. ahead, p. -, 2017. (14/00984-3, 15/21184-8)

PEDROSO, GISELE A.; KIMURA, ELZA M.; SANTOS, MAGNUN N. N.; ALBUQUERQUE, DULCINEIA M.; FERRUZZI, JUCILANE L. H.; JORGE, SUSAN E.; COSTA, FERNANDO F.; SAAD, SARA T. O.; SONATI, MARIA F.. Coinheritance of Hb Bristol-Alesha [beta 67(E11)Val -> Met; HBB: c.202G > A] and the alpha 212 Patchwork Allele in a Brazilian Child with Severe Congenital Hemolytic Anemia. HEMOGLOBIN, v. 41, n. 3, p. 203-208, 2017. (14/00984-3, 15/13710-1)

OLATUNYA, OLADELE S.; ALBUQUERQUE, DULCINEA M.; FAGBAMIGBE, ADENIYI F.; FABOYA, OPEYEMI A.; AJIBOLA, AYOTUNDE E.; BABALOLA, OLUWATOYIN A.; ADEBISI, ADEWALE O.; FALUSI, ADEYINKA G.; ADEKILE, ADEKUNLE; COSTA, FERNANDO F.. Diagnostic Accuracy of HemotypeSC as a Point-of-Care Testing Device for Sickle Cell Disease: Findings from a Southwestern State in Nigeria and Implications for Patient Care in Resource-Poor Settings of sub-Saharan Africa. GLOBAL PEDIATRIC HEALTH, v. 8, MAY 2021. (14/00984-3)

BRITO, PAMELA L.; DOS SANTOS, ALISSON F.; CHWEIH, HANAN; FAVERO, MARIA E.; GOTARDO, ERICA M. F.; SILVA, JULIETE A. F.; LEONARDO, FLAVIA C.; FRANCO-PENTEADO, CARLA F.; DE OLIVEIRA, MARIANA G.; FERREIRA JR, WILSON A.; et al. Reduced blood pressure in sickle cell disease is associated with decreased angiotensin converting enzyme (ACE) activity and is not modulated by ACE inhibition. PLoS One, v. 17, n. 2, p. 19-pg., 2022-02-03. (17/03954-6, 14/00984-3, 15/04485-4)

BERTOZZO, VICTOR DE HAIDAR E.; COSTA, SUELI MATILDE DA SILVA; ITO, MIRTA TOMIE; DA CRUZ, PEDRO RODRIGUES SOUSA; SOUZA, BRUNO BATISTA; RIOS, VINICIUS MANDOLESI; VITURINO, MARINA GONCALVES MONTEIRO; DE CASTRO, JULIA NICOLIELLO PEREIRA; RODRIGUES, THIAGO ADALTON ROSA; LANARO, CAROLINA; et al. Comparative transcriptome analysis of endothelial progenitor cells of HbSS patients with and without proliferative retinopathy. Experimental Biology and Medicine, v. 248, n. 8, p. 8-pg., 2023-04-03. (14/00984-3, 15/14255-6, 19/18886-1)

GARCIA, FLAVIA; MENDONCA, RAFAELA; MIGUEL, LEDIANA I.; DOMINICAL, VENINA M.; SAAD, SARA T. O.; COSTA, FERNANDO F.; CONRAN, NICOLA. CXCR4(hi) effector neutrophils in sickle cell anemia: potential role for elevated circulating serotonin (5-HT) in CXCR4(hi) neutrophil polarization. SCIENTIFIC REPORTS, v. 10, n. 1, p. 11-pg., 2020-08-31. (14/00984-3, 15/07637-0)