The Experts below are selected from a list of 291 Experts worldwide ranked by ideXlab platform
Narla Mohandas - One of the best experts on this subject based on the ideXlab platform.
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hereditary spherocytosis elliptocytosis and other red Cell Membrane disorders
Blood Reviews, 2013Co-Authors: Lydie Da Costa, Julie Galimand, Odile Fenneteau, Narla MohandasAbstract:Hereditary spherocytosis and elliptocytosis are the two most common inherited red Cell Membrane disorders resulting from mutations in genes encoding various red Cell Membrane and skeletal proteins. Red Cell Membrane, a composite structure composed of lipid bilayer linked to spectrin-based Membrane skeleton is responsible for the unique features of flexibility and mechanical stability of the Cell. Defects in various proteins involved in linking the lipid bilayer to Membrane skeleton result in loss in Membrane cohesion leading to surface area loss and hereditary spherocytosis while defects in proteins involved in lateral interactions of the spectrin-based skeleton lead to decreased mechanical stability, Membrane fragmentation and hereditary elliptocytosis. The disease severity is primarily dependent on the extent of Membrane surface area loss. Both these diseases can be readily diagnosed by various laboratory approaches that include red blood Cell cytology, flow cytometry, ektacytometry, electrophoresis of the red Cell Membrane proteins, and mutational analysis of gene encoding red Cell Membrane proteins.
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red Cell Membrane past present and future
Blood, 2008Co-Authors: Narla Mohandas, Patrick G GallagherAbstract:As a result of natural selection driven by severe forms of malaria, 1 in 6 humans in the world, more than 1 billion people, are affected by red Cell abnormalities, making them the most common of the inherited disorders. The non-nucleated red Cell is unique among human Cell type in that the plasma Membrane, its only structural component, accounts for all of its diverse antigenic, transport, and mechanical characteristics. Our current concept of the red Cell Membrane envisions it as a composite structure in which a Membrane envelope composed of cholesterol and phospholipids is secured to an elastic network of skeletal proteins via transMembrane proteins. Structural and functional characterization of the many constituents of the red Cell Membrane, in conjunction with biophysical and physiologic studies, has led to detailed description of the way in which the remarkable mechanical properties and other important characteristics of the red Cells arise, and of the manner in which they fail in disease states. Current studies in this very active and exciting field are continuing to produce new and unexpected revelations on the function of the red Cell Membrane and thus of the Cell in health and disease, and shed new light on Membrane function in other diverse Cell types.
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disorders of red Cell Membrane
British Journal of Haematology, 2008Co-Authors: Xiuli An, Narla MohandasAbstract:Studies during the last three decades have enabled the development of detailed molecular insights into the structural basis of altered function in various inherited red Cell Membrane disorders. This review highlights our current understanding of molecular and mechanistic insights into various inherited red Cell Membrane disorders involving either altered Membrane structural organization (hereditary spherocytosis, hereditary elliptocytosis and hereditary ovalocytosis) or altered Membrane transport function (hereditary stomatocytosis). The molecular basis for the vast majority of cases of hereditary spherocytosis, elliptocytosis and ovalocytosis have been fully defined while little progress has been made in defining the molecular basis for hereditary stomatocytosis. Mutations in a number of distinct genes account for hereditary spherocytosis and elliptocytosis, while a single genetic defect accounts for all cases of hereditary ovalocytosis. Based on these molecular insights, a comprehensive understanding of the structural basis for altered Membrane function has been developed. Loss of vertical linkage between Membrane skeleton and lipid bilayer leads to Membrane loss in hereditary spherocytosis, while weakening of lateral linkages between skeletal proteins leads to Membrane fragmentation and surface area loss in hereditary elliptocytosis. Importantly, the severity of anaemia in both these disorders is directly related to extent of Membrane surface area loss. Splenectomy results in amelioration of anaemia.
Zhiyou Wen - One of the best experts on this subject based on the ideXlab platform.
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Utilization of pyrolytic substrate by microalga Chlamydomonas reinhardtii: Cell Membrane property change as a response of the substrate toxicity
Applied Microbiology and Biotechnology, 2016Co-Authors: Xuefei Zhao, Laura Jarboe, Zhiyou WenAbstract:© 2016, Springer-Verlag Berlin Heidelberg.Acetic acid derived from fast pyrolysis of lignoCellulosic biomass is a promising substrate for microalgae fermentation for producing lipid-rich biomass. However, crude pyrolytic acetic acid solution contains various toxic compounds inhibiting algal growth. It was hypothesized that such an inhibition was mainly due to the Cell Membrane damage. In this work, the Cell Membrane property of algal Cells was evaluated at various conditions to elucidate the mechanisms of inhibition caused by the pyrolytic substrate solution. It was found that acetic acid itself served a carbon source for boosting algal Cell growth but also caused Cell Membrane leakage. The acetic acid concentration for highest Cell density was 4 g/L. Over-liming treatment of crude pyrolytic acetic acid increased the algal growth with a concurrent reduction of Cell Membrane leakage. Directed evolution of algal strain enhanced Cell Membrane integrity and thus increased its tolerance to the toxicity of the crude substrate. Statistical analysis shows that there was a significant correlation between the Cell growth performance and the Cell Membrane integrity (leakage) but not Membrane fluidity. The addition of cyto-protectants such as Pluronic F68 and Pluronic F127 enhanced the Cell Membrane integrity and thus, resulted in enhanced Cell growth. The transmission electron microscopy (TEM) of algal Cells visually confirmed the Cell Membrane damage as the mechanism of the pyrolytic substrate inhibition. Collectively, this work indicates that the Cell Membrane is one major reason for the toxicity of pyrolytic acetic acid when being used for algal culture. To better use this pyrolytic substrate, Cell Membrane of the microorganism needs to be strengthened through either strain improvement or addition of Membrane protectant reagents.
Patrick G Gallagher - One of the best experts on this subject based on the ideXlab platform.
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red Cell Membrane past present and future
Blood, 2008Co-Authors: Narla Mohandas, Patrick G GallagherAbstract:As a result of natural selection driven by severe forms of malaria, 1 in 6 humans in the world, more than 1 billion people, are affected by red Cell abnormalities, making them the most common of the inherited disorders. The non-nucleated red Cell is unique among human Cell type in that the plasma Membrane, its only structural component, accounts for all of its diverse antigenic, transport, and mechanical characteristics. Our current concept of the red Cell Membrane envisions it as a composite structure in which a Membrane envelope composed of cholesterol and phospholipids is secured to an elastic network of skeletal proteins via transMembrane proteins. Structural and functional characterization of the many constituents of the red Cell Membrane, in conjunction with biophysical and physiologic studies, has led to detailed description of the way in which the remarkable mechanical properties and other important characteristics of the red Cells arise, and of the manner in which they fail in disease states. Current studies in this very active and exciting field are continuing to produce new and unexpected revelations on the function of the red Cell Membrane and thus of the Cell in health and disease, and shed new light on Membrane function in other diverse Cell types.
Lydie Da Costa - One of the best experts on this subject based on the ideXlab platform.
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hereditary spherocytosis elliptocytosis and other red Cell Membrane disorders
Blood Reviews, 2013Co-Authors: Lydie Da Costa, Julie Galimand, Odile Fenneteau, Narla MohandasAbstract:Hereditary spherocytosis and elliptocytosis are the two most common inherited red Cell Membrane disorders resulting from mutations in genes encoding various red Cell Membrane and skeletal proteins. Red Cell Membrane, a composite structure composed of lipid bilayer linked to spectrin-based Membrane skeleton is responsible for the unique features of flexibility and mechanical stability of the Cell. Defects in various proteins involved in linking the lipid bilayer to Membrane skeleton result in loss in Membrane cohesion leading to surface area loss and hereditary spherocytosis while defects in proteins involved in lateral interactions of the spectrin-based skeleton lead to decreased mechanical stability, Membrane fragmentation and hereditary elliptocytosis. The disease severity is primarily dependent on the extent of Membrane surface area loss. Both these diseases can be readily diagnosed by various laboratory approaches that include red blood Cell cytology, flow cytometry, ektacytometry, electrophoresis of the red Cell Membrane proteins, and mutational analysis of gene encoding red Cell Membrane proteins.
Xiuli An - One of the best experts on this subject based on the ideXlab platform.
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disorders of red Cell Membrane
British Journal of Haematology, 2008Co-Authors: Xiuli An, Narla MohandasAbstract:Studies during the last three decades have enabled the development of detailed molecular insights into the structural basis of altered function in various inherited red Cell Membrane disorders. This review highlights our current understanding of molecular and mechanistic insights into various inherited red Cell Membrane disorders involving either altered Membrane structural organization (hereditary spherocytosis, hereditary elliptocytosis and hereditary ovalocytosis) or altered Membrane transport function (hereditary stomatocytosis). The molecular basis for the vast majority of cases of hereditary spherocytosis, elliptocytosis and ovalocytosis have been fully defined while little progress has been made in defining the molecular basis for hereditary stomatocytosis. Mutations in a number of distinct genes account for hereditary spherocytosis and elliptocytosis, while a single genetic defect accounts for all cases of hereditary ovalocytosis. Based on these molecular insights, a comprehensive understanding of the structural basis for altered Membrane function has been developed. Loss of vertical linkage between Membrane skeleton and lipid bilayer leads to Membrane loss in hereditary spherocytosis, while weakening of lateral linkages between skeletal proteins leads to Membrane fragmentation and surface area loss in hereditary elliptocytosis. Importantly, the severity of anaemia in both these disorders is directly related to extent of Membrane surface area loss. Splenectomy results in amelioration of anaemia.