hemoglobinopathies/phosphatase

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Sequential endoscopic/laparoscopic management of sickle hemoglobinopathy-associated cholelithiasis and suspected choledocholithiasis.

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We reviewed the medical records of 17 patients with sickle hemoglobinopathy-associated cholelithiasis who were candidates for laparoscopic cholecystectomy (LC) between 1991 and 1994. Eight patients with suspected choledocholithiasis (CDL) were identified, all of whom had preoperative endoscopic

Increased tyrosine phosphorylation of band 3 in hemoglobinopathies.

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In order to investigate the tyrosine phosphorylation of band 3, we performed immunoblotting of intact red cells using anti-phosphotyrosine antibody of 21 patients with sickle cell disorders (11 SS, 5 Sbeta, 5 SC), 7 patients with beta thalassemias (5 beta thal intermedia, 2 deltabeta thal), 10

Involvement of phosphatases in proliferation, maturation, and hemoglobinization of developing erythroid cells.

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Production of RBCs is triggered by the action of erythropoietin (Epo) through its binding to surface receptors (Epo-R) on erythroid precursors in the bone marrow. The intensity and the duration of the Epo signal are regulated by several factors, including the balance between the activities of

Therapeutic approach guided by genetic alteration: use of MTOR inhibitor in renal medullary carcinoma with loss of PTEN expression.

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Renal Medullary Cancer (RMC) is a rare and aggressive type of renal cell cancer that presents predominantly in patients with sickle cell hemoglobinopathies, and is typically metastatic at the time of presentation. Although platinum based chemotherapeutic regimens have recently emerged as the best

Erythrocyte K-Cl cotransport: properties and regulation.

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Erythrocytes possess a Cl-dependent, Na-independent K transport system cotransporting K and Cl in a 1:1 stoichiometry that is membrane potential independent. This K-Cl cotransporter is stimulated by cell swelling, acidification, Mg depletion, and thiol modification. Cell shrinkage, elevation of

Zinc status of children with sickle cell disease: relationship to poor growth.

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We examined the zinc status of 80 children with sickle cell disease (SCD) and 44 disease-free sibling controls aged 3 to 18 years. For both patients and controls, variations in serum zinc by age, type of hemoglobinopathy, and growth status were measured. The mean serum zinc concentration of patients

Origin and utility of the reverse dot-blot.

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Reverse allele specific oligonucleotide assays provide a robust method for the molecular characterization of high-mutation spectrum disorders. Commercial test have been developed for human leukocyte antigens class I and class II regions of human chromosome 6, the cystic fibrosis transmembrane

Vanadate elevates fetal hemoglobin in human erythroid precursors by inhibiting cell maturation.

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Increased fetal hemoglobin (HbF) in erythroid precursors of patients with beta-hemoglobinopathies (sickle cell anemia and beta-thalassemia), in which adult hemoglobin synthesis is defective, ameliorates the clinical symptoms of the underlying diseases. The production of erythroid precursors depends

Icariin protects against iron overload-induced bone loss via suppressing oxidative stress.

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Iron overload is common in patients with diseases such as hemoglobinopathies, hereditary hemochromatosis or elderly men and postmenopausal women. This disorder is frequently associated with bone loss and recently has been considered as an independent risk factor for osteoporosis. By excess reactive

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