Flow cytometric analysis of eosin-5′-maleimide-labeled red blood cells has been proposed as a new method of identifying hereditary spherocytosis (HS). The aim of the present study was to analyze sensitivity and specificity of this method. Red blood cells from patients with HS (n = 58) revealed significantly lower mean channel fluorescence values than red blood cells from normal subjects (n = 110), unaffected HS family members (n = 8), and patients with other anemias (n = 44). Taking a mean channel fluorescence of 400.0 units as the threshold value identified by logistic regression, sensitivity and specificity of the test for HS were 96.6 and 99.1%, respectively. Flow cytometric analysis is a valuable screening test for the diagnosis of HS.
Cadherins are calcium-depending cell adhesion proteins that play critical roles in brain morphogenesis and wiring. They provide an adhesive code for the development of cortical layers, due to their homophilic interactions and their restricted spatiotemporal expression patterns. In the adult organism, cadherins are involved in the maintenance and plasticity of neuronal circuits that play a role in learning. A well-known model for studying corticogenesis is the reeler mouse model. Numerous investigations of neocortical development suggest that, in the reeler mutant mouse, the lack of the protein Reelin results in cell-type and region-dependent changes of the neocortical layers. To investigate in detail how layer formation and regionalization is perturbed in the phylogenetically older archicortex of the adult reeler mutant mouse, we studied the expression of 11 different cadherins (Cdh4, Cdh7, Cdh8, Cdh11, Pcdh1, Pcdh7, Pcdh8, Pcdh9, Pcdh10, Pcdh17, and Pcdh19) and of the transcription factors ER81 and Cux2 by in situ hybridization in the (peri-)archicortex. All cadherins studied show a layer-specific expression in the (peri-)archicortex of the wildtype brain. In the archicortex of the reeler mutant, the cadherin-expressing cell layers are dispersed in the radial dimension, whereas in the periarchicortex the superficial and deep layers are inverted, both in the adult and during development. Possibly, this inversion relates to the histoarchitectural division of the reeler entorhinal cortex into an external and an internal zone. The regionalized, gradient-like expression of the cadherins is preserved in the reeler mutant mouse.
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