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Hot shape transformation: the role of PSar dehydration in stomatocyte morphogenesis.
Beilstein J Org Chem
January 2025
Institute of Molecules and Materials, Radboud University, Heyendaalseweg 135, 6525 AJ, Nijmegen, The Netherlands.
Polysarcosine emerges as a promising alternative to polyethylene glycol (PEG) in biomedical applications, boasting advantages in biocompatibility and degradability. While the self-assembly behavior of block copolymers containing polysarcosine-containing polymers has been reported, their potential for shape transformation remains largely untapped, limiting their versatility across various applications. In this study, we present a comprehensive methodology for synthesizing, self-assembling, and transforming polysarcosine-poly(benzyl glutamate) block copolymers, resulting in the formation of bowl-shaped vesicles, disks, and stomatocytes.
View Article and Find Full Text PDFEHBP1L1, an apicobasal polarity regulator, is critical for nuclear polarization during enucleation of erythroblasts.
Blood Adv
July 2023
Department of Cell Biology, Graduate School of Medicine, Osaka University, Suita, Japan.
Cell polarity, the asymmetric distribution of proteins and organelles, is permanently or transiently established in various cell types and plays an important role in many physiological events. epidermal growth factor receptor substrate 15 homology domain-binding protein 1-like 1 (EHBP1L1) is an adapter protein that is localized on recycling endosomes and regulates apical-directed transport in polarized epithelial cells. However, the role of EHBP1L1 in nonepithelial cells, remains unknown.
View Article and Find Full Text PDFJuvenile Hemochromatosis With Non-transfused Hemolytic Anemia Caused by a De Novo PIEZO1 Gene Mutation.
J Pediatr Hematol Oncol
May 2023
Department of Transfusion Medicine and Cell Processing, Tokyo Women's Medical University, Japan.
Article Synopsis
- A 23-year-old woman was diagnosed with macrocytic hemolytic anemia and iron overload, making her case complex to classify.
- Key lab findings included high serum ferritin and transferrin saturation, alongside low serum transferrin and ceruloplasmin.
- Genetic testing revealed a new mutation in the PIEZO1 gene, previously linked to dehydrated hereditary stomatocytosis, emphasizing the need to consider this condition when diagnosing iron overload with hemolytic anemia in younger patients.
A novel PIEZO1 mutation in a patient with dehydrated hereditary stomatocytosis: a case report and a brief review of literature.
Ital J Pediatr
July 2020
Department of Pediatrics, "Lalla Seràgnoli," Hematology-Oncology Unit, Sant'Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 11, 40137, Bologna, Italy.
Background: Dehydrated hereditary stomatocytosis (DHS) or hereditary xerocytosis is a rare, autosomal dominant hemolytic anemia characterized by macrocytosis, presence of stomatocytes and dehydration of red blood cells (RBCs). The dehydration is caused by a defect in cellular cation content. The most frequent expression of the pathology is hemolytic well-compensated anemia with high reticulocyte count, a tendency to macrocytosis, increased mean corpuscular hemoglobin concentration (MCHC) and mild jaundice.
View Article and Find Full Text PDFMild erythrocytosis as a presenting manifestation of associated erythrocyte volume disorders.
Pediatr Hematol Oncol
August 2019
Division of Pediatric Hematology and Oncology, Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Detroit , Michigan , USA.
Piezo1, encoded by the gene , is an erythrocytic cellular membrane mechanoactivated cation channel. Mutations have been implicated in erythrocyte volume disorders (EVDs)-especially hereditary xerocytosis (HX)/dehydrated stomatocytosis (DHS). We identified three patients, all with novel mutations, but only one displaying the HX/DHS phenotype.
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