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Impaired neutrophil chemotaxis in Pelger-Huët anomaly. 1979

H Repo, and P Vuopio, and M Leirisalo, and S E Jansson, and T U Kosunen

A family has previously been described in which four members with Pelger-Huët (P-H) anomaly suffered from recurrent attacks of abdominal pain and fever, while one member, whose polymorphonuclear leucocytes (PMNs) were also hyposegmented, was asymptomatic. We studied chemotaxis, chemokinesis and spontaneous locomotion of PMNs in the three surviving symptomatic sisters, in their asymptomatic brother and in two asymptomatic members of another family with P-H anomaly. The spontaneous migration of the PMNs of the three sisters was significantly slower both under agarose and in a membrane filter than that of the PMNs of the asymptomatic patients with P-H anomaly. Chemotactic and chemokinetic locomotion of the PMNs of the symptomatic sisters was also slow. Our results suggests that the impaired chemotaxis was due to a defect in the intrinsic locomotor capacity of PMNs rather than in their deformability or their responsiveness to the chemotactic stimulus.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009504 Neutrophils Granular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes. LE Cells,Leukocytes, Polymorphonuclear,Polymorphonuclear Leukocytes,Polymorphonuclear Neutrophils,Neutrophil Band Cells,Band Cell, Neutrophil,Cell, LE,LE Cell,Leukocyte, Polymorphonuclear,Neutrophil,Neutrophil Band Cell,Neutrophil, Polymorphonuclear,Polymorphonuclear Leukocyte,Polymorphonuclear Neutrophil
D010381 Pelger-Huet Anomaly Autosomal dominant anomaly characterized by abnormal ovoid shape GRANULOCYTE nuclei and their clumping chromatin. Mutations in the LAMIN B receptor gene that results in reduced protein levels are associated with the disorder. Heterozygote individuals are healthy with normal granulocyte function while homozygote individuals occasionally have skeletal anomalies, developmental delay, and seizures. Ovoid Neutrophil Nuclei, Developmental Delay, Epilepsy and Skeletal Abnormalities,Ovoid Neutrophil Nuclei, Developmental Delay, Epilepsy, and Skeletal Abnormalities,Pelger-Huet Nuclear Anomaly,Pelger-Huët Anomaly,Pelger-Huët Nuclear Anomaly,Pseudo Pelger-Huet Anomaly,Pseudo Pelger-Huet Nuclear Anomaly,Pseudo Pelger-Huët Anomaly,Anomaly, Pelger-Huet,Anomaly, Pelger-Huet Nuclear,Anomaly, Pelger-Huët,Anomaly, Pelger-Huët Nuclear,Anomaly, Pseudo Pelger-Huet,Anomaly, Pseudo Pelger-Huët,Nuclear Anomaly, Pelger-Huet,Nuclear Anomaly, Pelger-Huët,Pelger Huet Anomaly,Pelger Huet Nuclear Anomaly,Pelger Huët Anomaly,Pelger Huët Nuclear Anomaly,Pelger-Huet Anomaly, Pseudo,Pelger-Huët Anomaly, Pseudo,Pseudo Pelger Huet Anomaly,Pseudo Pelger Huet Nuclear Anomaly,Pseudo Pelger Huët Anomaly
D002465 Cell Movement The movement of cells from one location to another. Distinguish from CYTOKINESIS which is the process of dividing the CYTOPLASM of a cell. Cell Migration,Locomotion, Cell,Migration, Cell,Motility, Cell,Movement, Cell,Cell Locomotion,Cell Motility,Cell Movements,Movements, Cell
D002634 Chemotaxis, Leukocyte The movement of leukocytes in response to a chemical concentration gradient or to products formed in an immunologic reaction. Leukotaxis,Leukocyte Chemotaxis
D003470 Culture Media Any liquid or solid preparation made specifically for the growth, storage, or transport of microorganisms or other types of cells. The variety of media that exist allow for the culturing of specific microorganisms and cell types, such as differential media, selective media, test media, and defined media. Solid media consist of liquid media that have been solidified with an agent such as AGAR or GELATIN. Media, Culture
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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