Results 21 to 30 of about 429,758 (251)

Editing a γ-globin repressor binding site restores fetal hemoglobin synthesis and corrects the sickle cell disease phenotype

Science Advances, 2020
Editing the fetal γ-globin promoters in hematopoietic stem cells from sickle cell disease patients induces therapeutic γ-globin levels. Sickle cell disease (SCD) is caused by a single amino acid change in the adult hemoglobin (Hb) β chain that causes Hb ...
Leslie Weber, G. Frati, T. Felix, G. Hardouin, A. Casini, Clara Wollenschlaeger, Vasco Meneghini, C. Masson, A. de Cian, A. Chalumeau, F. Mavilio, Mario Amendola, I. André-Schmutz, A. Cereseto, W. El Nemer, J. Concordet, C. Giovannangeli, M. Cavazzana, A. Miccio   +18 more
semanticscholar   +1 more source

The growth of sickle hemoglobin polymers. [PDF]

Biophysical Journal, 2011
The measurement of polymer growth is an essential element in characterization of assembly. We have developed a precise method of measuring the growth of sickle hemoglobin polymers by observing the time required for polymers to traverse a photolytically produced channel between a region in which polymers are created and a detection region.
A. Aprelev, Zeng-Hui Liu, F. Ferrone
semanticscholar   +3 more sources

Allosteric control of hemoglobin S fiber formation by oxygen and its relation to the pathophysiology of sickle cell disease

Proceedings of the National Academy of Sciences of the United States of America, 2020
Significance The root cause of pathology in sickle cell disease is the polymerization of the mutant hemoglobin S upon deoxygenation in the tissues to form fibers.
E. Henry, Troy Cellmer, Emily B. Dunkelberger, Belhu B. Metaferia, J. Hofrichter, Quan Li, D. Ostrowski, R. Ghirlando, J. Louis, S. Moutéreau, F. Galactéros, S. Thein, P. Bartolucci, W. Eaton   +13 more
semanticscholar   +1 more source

Sickle Hemoglobin: A Specific Radioimmunoassay [PDF]

Blood, 1974
Abstract For the quantitation of hemoglobin S, a radioimmunoassay has been developed which is specific and highly sensitive. Hemoglobin S was purified by column chromatography and injected with complete Freund’s adjuvant into goats.
P T, Rowley, R A, Doherty, C, Rosecrans, E, Cernichiari   +3 more
openaire   +2 more sources

Hematologic and hemorheological determinants of resting and exercise-induced hemoglobin oxygen desaturation in children with sickle cell disease

Haematologica, 2013
The aim of the study was to determine the factors associated with resting and exercise-induced hemoglobin oxygen desaturation. The well-established six-minute walk test was conducted in 107 sickle cell children (50 with sickle hemoglobin C disease and 57
Xavier Waltz, Marc Romana, Marie-Laure Lalanne-Mistrih, Roberto F. Machado, Yann Lamarre, Vanessa Tarer, Marie-Dominique Hardy-Dessources, Benoît Tressières, Lydia Divialle-Doumdo, Marie Petras, Frederic Maillard, Maryse Etienne-Julan, Philippe Connes   +12 more
doaj   +1 more source

Study on HemoTypeSC™ test for the rapid screening of sickle cell disease patients in Government Medical College and Hospital, Ambikapur

Asian Journal of Medical Sciences, 2022
Background: Sickle cell disease (SCD), also known as sickle cell anemia, is a dangerous disease in which the body produces an abnormal form of hemoglobin, the protein in red blood cells that transport oxygen throughout the body. The body produces sickle-
Salma Mahaboob R , Obulesu G , Rajesh Kumar Srivastava , Murthy R , Parmanand Agarwal , Arya R C   +5 more
doaj   +1 more source

PF-07059013: A Noncovalent Modulator of Hemoglobin for Treatment of Sickle Cell Disease.

Journal of Medicinal Chemistry, 2020
Sickle cell disease (SCD) is a genetic disorder caused by a single point mutation (β6 Glu → Val) on the β-chain of adult hemoglobin (HbA) that results in sickled hemoglobin (HbS).
A. Gopalsamy, Ann E. Aulabaugh, Amey Barakat, K. Beaumont, Shawn Cabral, Daniel P. Canterbury, A. Casimiro-Garcia, Jeanne S. Chang, Ming Z. Chen, Chulho Choi, R. Dow, O. Fadeyi, Xidong Feng, Scott P. France, Roger M. Howard, Jay M. Janz, Jayasankar Jasti, R. Jasuja, L. Jones, Amanda J. King-Ahmad, Kelly M. Knee, J. Kohrt, C. Limberakis, S. Liras, Carlos A. Martinez, K. Mcclure, A. Narayanan, J. Narula, Jonathan J. Novak, Thomas N. O'Connell, Mihir D. Parikh, D. Piotrowski, O. Plotnikova, Ralph P. Robinson, P. Sahasrabudhe, Raman K. Sharma, B. Thuma, Dipy M. Vasa, Liu-Qing Wei, A. Wenzel, J. Withka, Jun Xiao, Hatice G. Yayla   +42 more
semanticscholar   +1 more source

Perspective: A novel prognostic for sickle cell disease

Saudi Journal of Medicine and Medical Sciences, 2018
Sickle hemoglobin (α2βS2) polymerization drives disease pathophysiology in sickle cell anemia. Fetal hemoglobin (α2γ2) restricts disease severity by inhibiting the polymerization of sickle hemoglobin in a concentration-dependent manner. Clinical decision-
Brian M Mozeleski, Abdullah Al-Rubaish, Amein Al-Ali, Jose Romero   +3 more
doaj   +1 more source

Targeting sickle cell disease root-cause pathophysiology with small molecules

Haematologica, 2019
The complex, frequently devastating, multi-organ pathophysiology of sickle cell disease has a single root cause: polymerization of deoxygenated sickle hemoglobin.
Yogen Saunthararajah
doaj   +1 more source

The spectrum of sickle hemoglobin-related nephropathy: from sickle cell disease to sickle trait

Expert Review of Hematology, 2017
Introduction: Renal dysfunction is among the most common complication of sickle cell disease (SCD), from hyposthenuria in children to progression to overt chronic kidney disease (CKD) in young adults. Emerging evidence now suggests that sickle hemoglobin-
Rakhi P. Naik, V. Derebail
semanticscholar   +1 more source