|
Thalassemia
|
0.100 |
Biomarker
|
group |
BEFREE |
It has previously been demonstrated that the self-inactivating γ-globin lentiviral vector GGHI can significantly increase fetal hemoglobin (HbF) in erythroid cells from thalassemia patients and thus improve the disease phenotype <i>in vitro.</i> In the present study, the GGHI vector was improved further by incorporating novel enhancer elements and also pseudotyping it with the baboon endogenous virus envelope glycoprotein BaEVRless, which efficiently and specifically targets human CD34<sup>+</sup> cells.
|
30324804 |
2019 |
|
Thalassemia
|
0.100 |
Biomarker
|
group |
BEFREE |
Those that produce abnormal hemoglobin are called structural hemoglobinopathies while thalassemia is another type of disorder that is caused by a defect in the gene production of the globin chains.
|
31025160 |
2019 |
|
Thalassemia
|
0.100 |
AlteredExpression
|
group |
BEFREE |
Recently, resveratrol showed induction of γ-globin mRNA synthesis in human erythroid precursors and reducing oxidative stress in red cells of thalassemia patients in many in vitro studies.
|
29926158 |
2018 |
|
Thalassemia
|
0.100 |
Biomarker
|
group |
BEFREE |
Hereditary persistence of fetal hemoglobin deletion type-2 (HPFH-2) and Sicilian-δβ-thalassemia are conditions described as large deletions of the human β-like globin cluster, with absent β-globin chains and a compensatory variable increase in γ-globin.
|
27591578 |
2017 |
|
Thalassemia
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Heterogeneity in thalassemia is due to various modifying factors viz. coinheritance of α-gene defects, abnormal hemoglobin, XmnI polymorphism, variation in repeat sequences present in LCR, and silencer region of the gene.
|
25976460 |
2015 |
|
Thalassemia
|
0.100 |
GeneticVariation
|
group |
BEFREE |
These results, without undermining the strength of BCL11A as a silencer of the γ globin gene, suggest that the LCR background, by governing the state of BCL11A binding to this region, plays a more significant role in determining the thalassemia phenotype than the level of BCL11A protein expression, that might be influenced by single nucleotide polymorphisms in intronic regions of the BCL11A gene.
|
23541515 |
2013 |
|
Thalassemia
|
0.100 |
Biomarker
|
group |
BEFREE |
Frequency of the G allele in the polymorphic palindromic sequence of 5'HS4 (TGGGG A/G CCCCA) and positive Xmn1-HBG2 profile was significantly higher in thalassemia patients compared to the normal population.
|
22036762 |
2012 |
|
Thalassemia
|
0.100 |
Biomarker
|
group |
BEFREE |
Thalassemia and abnormal hemoglobin are the most common genetic disorders and are considered health problems in many developing countries.
|
22762348 |
2012 |
|
Thalassemia
|
0.100 |
AlteredExpression
|
group |
BEFREE |
Understanding the regulation of the human γ-globin gene expression is a challenge for the treatment of thalassemia.
|
23272095 |
2012 |
|
Thalassemia
|
0.100 |
GeneticVariation
|
group |
BEFREE |
The Corfu deltabeta thalassemia deletion disrupts gamma-globin gene silencing and reveals post-transcriptional regulation of HbF expression.
|
15536151 |
2005 |
|
Thalassemia
|
0.100 |
Biomarker
|
group |
BEFREE |
In early studies, 5-azacytidine produced significant HbF elevations in patients with thalassemia and SCD, but clinical development of this class of agent was halted after a poorly controlled animal study suggested that 5-azacytidine might be carcinogenic.
|
15534852 |
2004 |
|
Thalassemia
|
0.100 |
Biomarker
|
group |
BEFREE |
Thalassemia and abnormal hemoglobin.
|
12430905 |
2002 |
|
Thalassemia
|
0.100 |
AlteredExpression
|
group |
BEFREE |
Deletion of a region that is a candidate for the difference between the deletion forms of hereditary persistence of fetal hemoglobin and deltabeta-thalassemia affects beta- but not gamma-globin gene expression.
|
10022837 |
1999 |
|
Thalassemia
|
0.100 |
AlteredExpression
|
group |
BEFREE |
Maximal gamma-globin expression in the compound heterozygous state for -175G gamma HPFH and beta degree 39 nonsense thalassaemia: a case study.
|
9222287 |
1997 |
|
Thalassemia
|
0.100 |
AlteredExpression
|
group |
BEFREE |
Low G gamma-globin expression in the adult is associated with two haplotypes that are not common between thalassemia and sickle cell anemia patients.
|
2580306 |
1985 |
|
Thalassemia
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Correlation of haematological data and the location of deletions in two cases of HPFH and one case of deltabeta-thalassaemia suggest that a region of DNA located near the 5'-end of the delta-globin gene may be involved in the suppression in cis of gamma-globin gene expression in adults.
|
450109 |
1979 |