A 18-month-old girl with a diagnosis of Chediak-Higashi syndrome (CHS) presented with severe bacterial skin infection. Recurrent episodes of bacterial infections began at the age of 2 months. On physical examination, generalized lymphadenopathy, hepatosplenomegaly, skin hypopigmentation, and silvery hair were noted. Bone marrow aspirate revealed prominent granules (top left and right) within th...

The Chediak-Higashi syndrome (CHS) trait is expressed in cultured human skin fibroblasts as an abnormal perinuclear concentration of moderately enlarged lysosomes. The cytoskeleton of CHS fibroblasts appears intact. Microtubules are normal in number and morphology, as assessed by colchicine binding studies, antitubulin immunofluorescence, and electron microscopy. Deformability by shear force is...

Recent ERP findings challenge the widespread assumption that syntactic and semantic processes are tightly coupled. Syntactically well-formed sentences that are semantically anomalous due to thematic mismatches elicit a P600, the component standardly associated with syntactic anomaly. This 'thematic P600' effect has been attributed to detection of semantically plausible thematic relations that c...

1 Departamento de Microbiologı́a I, Inmunologı́a, Facultad de Medicina, Universidad Complutense, 28040 Madrid, Spain 2 Inmunotek SL, Alcalá de Henares, 28805 Madrid, Spain 3 School of Life and Health Sciences, Aston University, Birmingham B4 7ET, UK 4ATR, LLC, Worcester, MA 01606, USA 5Department of Mycobacteriology, National Institute of Infectious Diseases, Higashi-Murayama, Tokyo 189-0002, Japan

Graduate School of Integrated Arts and Sciences, Hiroshima University, Higashi-Hiroshima, Hiroshima 739-8521 Frontier Research System, The Institute of Physical and Chemical Research (RIKEN), Wako, Saitama 351-0198 AdSM, Hiroshima University, Higashi-Hiroshima, Hiroshima 739-8530 Center for Theoretical Physics, Physics Department, CSCS, The University of Michigan, Ann Arbor, MI 48109-1040, U.S....

An increased membrane fluidity was also detected with a spin label that probes deeper in the bilayer. In vitro treatment of Chediak-Higashi mouse cells with 0.01 M ascorbate increased the order parameter to normal levels. In vitro incubation of mouse Chediak-Higashi cells with glucose oxidase increased the order parameter, similar to the effect of ascorbate. This increase was abolished when cat...