نتایج جستجو برای: hbf

تعداد نتایج: 869  

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...

ژورنال: :فصلنامه پژوهشی خون 0
فیض اله هاشمی گرجی f. hashemi-gorji مرکز تحقیقات بیوتکنولوژی انستیتو پاستور ایران محمد حمید m. hamid مرکز تحقیقات بیوتکنولوژی انستیتو پاستور ایران ـسازمان اصلی تایید شده: انستیتو پاستور ایران (pasteur institute of iran) آیدا عرب a. arab مرکز تحقیقات بیوتکنولوژی انستیتو پاستور ایرانسازمان اصلی تایید شده: انستیتو پاستور ایران (pasteur institute of iran) اعظم امیریان a. amirian مرکز تحقیقات بیوتکنولوژی انستیتو پاستور ایرانسازمان اصلی تایید شده: انستیتو پاستور ایران (pasteur institute of iran) سیروس زینلی s. zeinali مرکز تحقیقات بیوتکنولوژی انستیتو پاستور ایرانسازمان اصلی تایید شده: انستیتو پاستور ایران (pasteur institute of iran) مرتضی کریمی پور m. karimipoor مرکز تحقیقات بیوتکنولوژی انستیتو پاستور ایرانسازمان اصلی تایید شده: انستیتو پاستور ایران (pasteur institute of iran)

چکید ه   سابقه و هدف   سطح بالای هموگلوبین جنینی (hbf) تأثیر عمده ای بر تالاسمی بتا دارد به طوری که افزایش تولید hbf ، شدت بیماری را کاهش می دهد. سه لوکوس hbs1l–myb در کروموزوم 6q23 ، bcl11a در کروموزوم 2p16 و ژن گاماگلوبین با افزایش سطح hbf در بیماران مبتلا به تالاسمی در ارتباط می باشند . در مطالعه کنونی، ارتباط برخی پلی مورفیسم های افزایش دهنده سطح hbf در بیماران مبتلا به تالاسمی و افراد سالم...

Journal: :international journal of hematology-oncology and stem cell research 0
mohammad ali jalali far health research institute, research center of thalassemia & hemoglobinopathy, ahvaz jundishapur university of medical sciences, ahvaz, iran ali dehghani fard sarem cell research center-scrc, sarem women’s hospital, tehran, iran saiedeh hajizamani diagnosticlaboratory sciences and technology research center, school of paramedical sciences, shiraz university of medical sciences, shiraz, iran majid mossahebi-mohammadi department of hematology, faculty of medical sciences, tarbiat modares university, tehran, iran hamid yaghooti department of medical laboratory sciences, school of paramedicine, ahvaz jundishapur university of medical sciences, ahvaz, iran najmaldin saki health research institute, research center of thalassemia & hemoglobinopathy, ahvaz jundishapur university of medical sciences, ahvaz, iran

background: efficient induction of fetal hemoglobin (hbf) is considered as an effective therapeutic approach in beta thalassemia. hbf inducer agents can induce the expression of γ-globin gene and produce high levels of hbf via different epigenetic and molecular mechanisms. thalidomide and sodium butyrate are known as hbf inducer drugs. material and methods : cd133 + stem cells were isolated fro...

Journal: :Ghana medical journal 2015
C Antwi-Boasiako E Frimpong G K Ababio B Dzudzor I Ekem B Gyan N A Sodzi-Tettey D A Antwi

BACKGROUND Foetal haemoglobin has been implicated in the modulation of sickle cell crisis. Its level is generally inversely proportional to the severity of sickle cell disease (SCD) for a given sickle cell phenotypes. The main aim of therapy for vaso-occlusive crisis (VOC), which is the hallmark of SCD, is to reduce the chances of sickling through the prevention of polymerization of HbS. One wa...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2008
Manuela Uda Renzo Galanello Serena Sanna Guillaume Lettre Vijay G Sankaran Weimin Chen Gianluca Usala Fabio Busonero Andrea Maschio Giuseppe Albai Maria Grazia Piras Natascia Sestu Sandra Lai Mariano Dei Antonella Mulas Laura Crisponi Silvia Naitza Isadora Asunis Manila Deiana Ramaiah Nagaraja Lucia Perseu Stefania Satta Maria Dolores Cipollina Carla Sollaino Paolo Moi Joel N Hirschhorn Stuart H Orkin Gonçalo R Abecasis David Schlessinger Antonio Cao

beta-Thalassemia and sickle cell disease both display a great deal of phenotypic heterogeneity, despite being generally thought of as simple Mendelian diseases. The reasons for this are not well understood, although the level of fetal hemoglobin (HbF) is one well characterized ameliorating factor in both of these conditions. To better understand the genetic basis of this heterogeneity, we carri...

2012
Sanjana Bhagat Pradeep Kumar Patra Amar Singh Thakur

The γ(G)-158 (C→T) polymorphism plays important function in the disease severity of sickle cell anemia. The XmnI restriction site at -158 position of the γ(G)-gene is associated with increased expression of the γ(G)-globin gene and higher production of HbF. This study aims to determine the frequency of the different genotypes of the γ(G) Xmn I polymorphism in sickle cell anemia and sickle cell ...

2017
Cyril Cyrus Chittibabu Vatte J Francis Borgio Abdullah Al-Rubaish Shahanas Chathoth Zaki A Nasserullah Sana Al Jarrash Ahmed Sulaiman Hatem Qutub Hassan Alsaleem Alhusain J Alzahrani Martin H Steinberg Amein K Al Ali

Background and Objectives. β-Thalassemia and sickle cell disease are genetic disorders characterized by reduced and abnormal β-globin chain production, respectively. The elevation of fetal hemoglobin (HbF) can ameliorate the severity of these disorders. In sickle cell disease patients, the HbF level elevation is associated with three quantitative trait loci (QTLs), BCL11A, HBG2 promoter, and HB...

Journal: :Blood 2008
Rodwell Mabaera Michael R Greene Christine A Richardson Sarah J Conine Courtney D Kozul Christopher H Lowrey

5-azacytidine (5-Aza) is a potent inducer of fetal hemoglobin (HbF) in people with beta-thalassemia and sickle cell disease. Two models have been proposed to explain this activity. The first is based on the drug's ability to inhibit global DNA methylation, including the fetal globin genes, resulting in their activation. The second is based on 5-Aza's cytotoxicity and observations that HbF produ...

Journal: :Blood 1991
E P Economou S E Antonarakis H H Kazazian G R Serjeant G J Dover

Single nucleotide substitutions in the promoter regions of the A gamma- and G gamma-globin genes have been associated with increased fetal hemoglobin (HbF) production. We wished to determine whether these or other unrecognized substitutions in the gamma promoter regions are responsible for the 20-fold variation in HbF production in sickle cell patients or normal adults. From a random sampling o...

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 ...

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