The Experts below are selected from a list of 150 Experts worldwide ranked by ideXlab platform
Minoru Fukuda - One of the best experts on this subject based on the ideXlab platform.
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HempaS. Hereditary erythroblastic multinuclearity with positive acidified serum lysis test.
Biochimica et biophysica acta, 1999Co-Authors: Minoru FukudaAbstract:Congenital dyserythropoietic anemia type II or HempaS (hereditary erythroblastic multinuclearity with positive acidified serum lysis test) is a genetic anemia in humans caused by a glycosylation deficiency. Erythrocyte membrane glycoproteins, such as band 3 and band 4.5, which are normally glycosylated with polylactosamines lack these carbohydrates in HempaS. Polylactosamines accumulate as glycolipids in HempaS erythrocytes. Analysis of N-glycans from HempaS erythrocyte membranes revealed a series of incompletely processed N-glycan structures, indicating defective glycosylation at N-acetylglucosaminyltransferase II (GnT-II) and/or alpha-mannosidase II (MII) steps. Genetic analysis has identified two cases from England in which the MII gene is defective. Mutant mice in which the MII gene was inactivated by homologous recombination resulted in a HempaS-like phenotype. On the other hand, linkage analysis of HempaS cases from southern Italy excluded MII and GnT-II as the causative gene, but identified a gene on chromosome 20q11. HempaS is therefore genetically heterogeneous. Regardless of which gene is defective, HempaS is characterized by incomplete processing of N-glycans. The study of HempaS will identify hitherto unknown factors affecting N-glycan synthesis.
Pamela I. Ellsworth - One of the best experts on this subject based on the ideXlab platform.
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Priapism in a 15-year-old boy with congenital dyserythropoietic anemia type II (hereditary erythroblastic multinuclearity with positive acidified serum lysis test).
The Journal of urology, 2002Co-Authors: Mark T. Edney, Alan R. Schned, Marc Cendron, Sara Chaffee, Pamela I. EllsworthAbstract:Congenital dyserythropoietic anemia encompasses a group of related disorders characterized by ineffective erythropoiesis, bone marrow erythroblastic multinuclearity and secondary hemochromatosis. Type II, the most common variation, is called hereditary erythroblastic multinuclearity with positive acidified serum lysis test (HempaS). To our knowledge this is the first reported case of priapism associated with HempaS.
Gordon Port - One of the best experts on this subject based on the ideXlab platform.
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Two methods of estimating competitiveness of chemosterilized males of Musca domestica (Diptera: Muscidae)
Bulletin of Entomological Research, 1992Co-Authors: M. Saiful Islam, Gordon PortAbstract:Mating competitiveness and sexual or total competitiveness of males of the house fly, Musca domestica Linnaeus, treated with 1.0% hexamethyl phosphoramide (Hempa) were measured quantitatively from direct observations and ratio test methods, respectively. From direct observations, treated males of Cooper and Boxted strains of M. domestica were found to obtain more first matings than untreated males (P
Mark T. Edney - One of the best experts on this subject based on the ideXlab platform.
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Priapism in a 15-year-old boy with congenital dyserythropoietic anemia type II (hereditary erythroblastic multinuclearity with positive acidified serum lysis test).
The Journal of urology, 2002Co-Authors: Mark T. Edney, Alan R. Schned, Marc Cendron, Sara Chaffee, Pamela I. EllsworthAbstract:Congenital dyserythropoietic anemia encompasses a group of related disorders characterized by ineffective erythropoiesis, bone marrow erythroblastic multinuclearity and secondary hemochromatosis. Type II, the most common variation, is called hereditary erythroblastic multinuclearity with positive acidified serum lysis test (HempaS). To our knowledge this is the first reported case of priapism associated with HempaS.
M. Saiful Islam - One of the best experts on this subject based on the ideXlab platform.
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Two methods of estimating competitiveness of chemosterilized males of Musca domestica (Diptera: Muscidae)
Bulletin of Entomological Research, 1992Co-Authors: M. Saiful Islam, Gordon PortAbstract:Mating competitiveness and sexual or total competitiveness of males of the house fly, Musca domestica Linnaeus, treated with 1.0% hexamethyl phosphoramide (Hempa) were measured quantitatively from direct observations and ratio test methods, respectively. From direct observations, treated males of Cooper and Boxted strains of M. domestica were found to obtain more first matings than untreated males (P