نتایج جستجو برای: hereditary spherocytosis

تعداد نتایج: 84467  

Journal: :Blood 2011
Guillaume Deplaine Innocent Safeukui Fakhri Jeddi François Lacoste Valentine Brousse Sylvie Perrot Sylvestre Biligui Micheline Guillotte Corinne Guitton Safi Dokmak Béatrice Aussilhou Alain Sauvanet Dominique Cazals Hatem François Paye Marc Thellier Dominique Mazier Geneviève Milon Narla Mohandas Odile Mercereau-Puijalon Peter H David Pierre A Buffet

Retention of poorly deformable red blood cells (RBCs) by the human spleen has been recognized as a critical determinant of pathogenesis in hereditary spherocytosis, malaria, and other RBC disorders. Using an ex vivo perfusion system, we had previously shown that retention of Plasmodium falciparum-infected RBCs (Pf-RBCs) occur in the splenic red pulp, upstream from the sinus wall. To experimenta...

Journal: :Cellular Physiology and Biochemistry 2021

Background/Aims: Hereditary Spherocytosis (HS) is the most common erythrocyte membrane disorder causing hemolytic anemia. The wide heterogeneity of both clinical and laboratory manifestations HS contributes to difficulties associated with diagnosis this disorder. Although massive data previously reported worldwide, there yet no on among Tunisian population. Here we aim characterize in patients ...

Journal: :Blood 2004
Luanne L Peters Rebecca A Swearingen Sabra G Andersen Babette Gwynn Amy J Lambert Renhua Li Samuel E Lux Gary A Churchill

Defects in red blood cell (RBC) membrane skeleton components cause hereditary spherocytosis (HS). Clinically, HS varies significantly even among individuals with identical gene defects, illustrating the profound effects of genetic background on disease severity. We exploited a new spontaneous mouse model, wan, which arose on the inbred C3H/HeJ strain, to identify quantitative trait loci (QTL) t...

Journal: :Blood 1982
S R Goodman K A Shiffer L A Casoria M E Eyster

We have localized the molecular alteration in the membrane skeleton of two of four kindreds with hereditary spherocytosis (HS) to an alteration in the spectrin-protein-4.1 interaction due to a defective spectrin molecule. The defective spectrin-protein-4.1 interaction in these kindreds (referred to as type I HS) leads to a weakened spectrin-protein-4.1-actin ternary complex, which in turn may l...

نمودار تعداد نتایج جستجو در هر سال

با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید