نتایج جستجو برای: higashi syndrome
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Background. Chediak-Higashi syndrome (CHS) is a rare, autosomal recessive disorder characterized by oculocutaneous albinism, immunodeficiency, coagulopathy and late-onset, progressive neurological dysfunction. It also has an "accelerated phase" characterized by hemophagocytic lymphohistiocytosis (HLH). The disease is caused by mutations in the CHS1/LYST gene located on chromosome 1, which affec...
Studies have demonstrated significant heterogeneity in neutrophil granule morphology and physical density. Using cytochemical methods to localize peroxidase and vicinal glycol containing complex carbohydrates we examined the heterogeneity of neutrophil granules from intact human neutrophil granules in 13 isolated granule density fractions, calcium ionophore A23187 treated neutrophils and neutro...
Following biosynthesis, class II MHC molecules are transported through a lysosome-like compartment, where they acquire antigenic peptides for presentation to T cells at the cell surface. This compartment is characterized by the presence of HLA-DM, which catalyzes the peptide loading process. Here we report that the morphology and function of the class II loading compartment is affected in disea...
School of Science and Engineering, Kinki University, Higashi-Osaka 577-8502, Japan Department of Mathematics, Graduate School of Science, Hokkaido University, Sapporo 060-0810, Japan Research Institute for Mathematical Sciences, Kyoto University, Kyoto 606-8502, Japan Department of Mathematics, Graduate School of Science, Kyoto University, Kyoto 606-8502 Japan Hitachi Ltd., Asagaya-kita 2-13-2,...
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