Results 201 to 210 of about 429,758 (251)

Biologic and Clinical Efficacy of LentiGlobin for Sickle Cell Disease.

New England Journal of Medicine, 2021
BACKGROUND Sickle cell disease is characterized by the painful recurrence of vaso-occlusive events. Gene therapy with the use of LentiGlobin for sickle cell disease (bb1111; lovotibeglogene autotemcel) consists of autologous transplantation of ...
J. Kanter, M. Walters, L. Krishnamurti, M. Mapara, J. Kwiatkowski, Stacey Rifkin-Zenenberg, B. Aygun, Kimberly A. Kasow, F. J. Pierciey, Melissa Bonner, Alexandra L. Miller, Xinyan Zhang, Jessie Lynch, Dennis Kim, J. Ribeil, M. Asmal, S. Goyal, A. Thompson, J. Tisdale   +18 more
semanticscholar   +1 more source

On the assembly of sickle hemoglobin fascicles

Journal of Molecular Biology, 1989
Deoxyhemoglobin S fibers associate into bundles, or fascicles, that subsequently crystallize by a process of alignment and fusion. We have used electron microscopy to study the formation of fascicles and the changes in fiber packing that occur during the conversion of fascicles to crystals.
W A, McDade, B, Carragher, C A, Miller, R, Josephs   +3 more
openaire   +2 more sources

Sickle Cell Anemia and Fetal Hemoglobin

American Journal of the Medical Sciences, 1994
Fetal hemoglobin, the predominant hemoglobin of the fetus, is good for sickle cell anemia. This hemoglobin inhibits the polymerization of sickle hemoglobin. Clinical studies have shown that at any level of fetal hemoglobin, the more that is present, the better off is the patient.

exaly   +3 more sources

The interactions of sickle hemoglobin

Biochimie, 1972
Resume Les interactions entre molecules d'hemoglobine qui conduisent a la falciformation des erythrocytes, aux crises micro-emboliques et a l'anemie chronique sont dues a la presence de la desoxyhemoglobine S (α 2 a β 2 6Val ). Au cours de la desoxygenation la viscosite des solutions concentrees d'hemoglobine S augmente progressivement; il y a ...
openaire   +2 more sources

The oxygen affinity of sickle hemoglobin

Respiratory Physiology & Neurobiology, 2008
The right-shifted oxyhemoglobin dissociation curve of sickle cell disease (SCD) has been thought to result in abnormally low arterial oxygen saturation (S(o)(2)), even when oxygen partial pressure (P(o)(2)) is normal. However, without polymer formation (minimal under normoxic conditions), HbS oxygen affinity is normal.
Amgad, Abdu, José, Gómez-Márquez, Thomas K, Aldrich   +2 more
openaire   +2 more sources

Fetal Hemoglobin, Sickling, and Sickle Cell Disease

Advances in Pediatrics, 1990
Increased numbers of F cells and large amounts of Hb F/F cell appear to produce clinical benefit in rare variants of sickle cell disease and probably in more commonly encountered patients. Fetal hemoglobin interferes with polymerization of Hb S in vitro, but laboratory studies carried out with homogeneous hemoglobin solutions are inadequate models of ...
openaire   +2 more sources

Sickle Cell Hemoglobin Polymerization

1990
Publisher Summary The chapter describes the understanding of the physics and physical chemistry of sickle cell hemoglobin polymerization in solutions and in red cells. The polymerization of sickle cell hemoglobin has probably become the best understood of all protein self-assembly systems.
W A, Eaton, J, Hofrichter
openaire   +2 more sources

Exagamglogene Autotemcel for Severe Sickle Cell Disease.

New England Journal of Medicine
BACKGROUND Exagamglogene autotemcel (exa-cel) is a nonviral cell therapy designed to reactivate fetal hemoglobin synthesis by means of ex vivo clustered regularly interspaced short palindromic repeats (CRISPR)-Cas9 gene editing of autologous CD34 ...
H. Frangoul, F. Locatelli, Akshay Sharma, Monica Bhatia, M. Mapara, Lyndsay Molinari, Donna Wall, R. Liem, Paul Telfer, Ami J. Shah, Marina Cavazzana, S. Corbacioglu, D. Rondelli, Roland Meisel, Laurence Dedeken, S. Lobitz, M. de Montalembert, Martin H. Steinberg, Mark C Walters, Michael J. Eckrich, S. Imren, Laura Bower, C. Simard, Wei-Yu Zhou, F. Xuan, Phuong K Morrow, W. Hobbs, Stephan A. Grupp   +27 more
semanticscholar   +1 more source

Plasma Hemoglobin and Hemoglobin Fractions in Sickle Cell Crisis

American Journal of Clinical Pathology, 1971
The geometric mean of plasma hemoglobin concentrations assayed by a modified benzidine procedure in 14 patients with sickle cell anemia (S-S hemoglobin) during 18 painful crises was 8.5 mg. per 100 ml. as compared with 5.9 mg. per 100 ml. during quiescent periods and 0.3 mg. per 100 ml. in normal controls. On the first and second days of crisis, plasma
H N, Naumann, L W, Diggs, L, Barreras, B J, Williams   +3 more
openaire   +2 more sources

A Novel Sickle Hemoglobin: Hemoglobin S-South End

Journal of Pediatric Hematology/Oncology, 2004
Sickle hemoglobin (Hb S; beta Glu6Val) is due to an AGTG; beta Lys132Asn, AAA>AAC). When present alone, the beta Lys132Asn mutation has low oxygen affinity. Therefore, this mutation may enhance the polymerization of the Hb S variant. Furthermore, the variant hemoglobin mimics Hb A on high-pressure liquid chromatography, and its identity is not easily ...
Hong-Yuan, Luo, Adeboye H, Adewoye, Shawn H, Eung, Timothy P, Skelton, Karen, Quillen, Lillian, McMahon, Martin H, Steinberg, David H K, Chui   +7 more
openaire   +2 more sources