Competing functions encoded in the allergy-associated F(c)epsilonRIbeta gene

Allergic reactions are triggered via crosslinking of the high-affinity receptor for immunoglobulin E, F(c)epsilonRI. In humans, F(c)epsilonRI is expressed as a tetramer (alphabetagamma(2)) and a trimer (alphagamma(2)). The beta subunit is an amplifier of F(c)epsilonRI surface expression and signaling. Here, we show that as a consequence of alternative splicing, the F(c)epsilonRIbeta gene encodes two proteins with opposing and competing functions. One isoform is the full-length classical beta, the other a novel truncated form, beta(T). In contrast to beta, beta(T) prevents F(c)epsilonRI surface expression by inhibiting alpha chain maturation. Moreover, beta(T) competes with beta to control F(c)epsilonRI surface expression in vitro. We propose that the relative abundance of the products of the beta gene may control the level of F(c)epsilonRI surface expression and thereby influence susceptibility to allergic diseases.     

Aneurysm detection using one-bit correlation

Intercranial aneurysm present an especially serious clinical problem, since their rupture generally results in death or permanent brain damage. Recent work, conducted by the authors, indicated that this anomaly can be detected using spectral signature analysis. A low-cost turnkey noninvasive aneurysm-detection system is being tested for this purpose. Using polarity-coincident noise-added one-bit correlators, aneurysms have been successfully detected.     

Segmental jejunal lipomatosis--a rare cause of intestinal obstruction

A rare case of a segmental small intestinal (jejunal) lipomatosis is described. A 33-year-old male was admitted with a clinical diagnosis of an acute intestinal obstruction. A plain erect abdominal x-ray showed multiple air-fluid levels. On an exploratory laparotomy, a jejunojejunal intussusception was found secondary to a segmental submucosal lipomatosis. This was treated by a segmental resection and anastomosis, which resulted in a complete cure. Here we present this case with a review of the relevant literature.     

Defective apoptosis in lymphocytes and the role of IL-2 in autoimmune hematologic cytopenias

Fas-mediated signaling is important for lymphocyte elimination. We investigated lymphocytes for Fas-signaling defects in 20 pediatric patients with chronic hematologic autoimmunity. In 5 of 20 (25%), there was profound resistance to exogenous FasL-mediated lysis, Fas mAb, and anti-CD3. FasL function, though variable, was not significantly different from that of simultaneously evaluated controls. Only 1 patient had a Fas mutation and manifestations of autoimmune lymphoproliferative syndrome. In contrast, lymphocytes from his clinically normal mother with the same mutation were normally sensitive to FasL. In 3 patients, normal Fas-mediated lysis was restored with rhIL-2. IL-2 had no effect in the other 2 patients. Activation and proliferation functions of IL-2 were normal in all 5. We conclude that altered Fas signaling, independent of Fas mutations, can precipitate hematologic autoimmunity. IL-2 can rescue some lymphocytes from this defect. In IL-2 refractory cases, a persistently defective response to IL-2 continues to confer a lymphocyte survival advantage. Hence, altered Fas pathway signaling with or without defective IL-2 responses should be considered in the etiology of hematologic autoimmunity.     

Mature teratoma arising from the middle cranial fossa

A 25-year-old man presented with a sudden spurt in size of a bony swelling around the external ear, which was present since birth. This was associated with a painful swelling of the right half of the face and pain, redness, and decreased vision in the right eye. Neurologic examination showed decreased visual acuity in the right eye and paresis of the fifth, seventh, and eighth cranial nerves. Neuroimaging showed an extra-axial, partly cystic tumor of mixed density, located entirely within the middle cranial fossa scalloping the temporal bone and extending into the right infratemporal fossa and the right middle ear and impinging on the ipsilateral cavernous sinus. The right temporal lobe was elevated and compressed. Histopathologic examination confirmed a mature teratoma.     

Learning by Selection in the Trion Model of Cortical Organization

The basic issue of whether mammalian learning in cortex proceedsvia a selection principle, as stressed by Edelman, versus aninstructional one is of major importance. We present here arealization of selection learning in the trion model, whichis based on the Mountcastle columnar organizational principleof cortex. We suggest that mammalian cortex starts out withan a priori connectivity between minicolumns that is highlystructured in time and in space, competing between excitationand inhibition. This provides a "naive" repertoire of spatial-temporalfiring patterns that stimuli and internal pro-cessing map onto.These patterns can be learned with small modifications to theconnectivity strengths determined by a Hobbian learning rule.As various patterns are learned, the repertoire changes somewhatin order to respond property to various stimuli, but the majorityof all possible stimuli still map onto spatial-temporal firingpatterns of the original repertoire. In order to show that theexample presented here is showing true selectivity and is notan artifact of more stimuli evolving into the learned pattern,we develop a selectivity measure. We suggest that some formof instructional learning (in which connectivities are finelytuned) is present for difficult tasks requiring many trials,whereas very rapid learning involves selectional learning. Bothtypes of learning must be considered to understand behavior.