Enzyme replacement prevents enamel defects in hypophosphatasia mice

Hypophosphatasia (HPP) is the inborn error of metabolism characterized by deficiency of alkaline phosphatase activity, leading to rickets or osteomalacia and to dental defects. HPP occurs from loss-of-function mutations within the gene that encodes the tissue-nonspecific isozyme of alkaline phosphatase (TNAP). TNAP knockout (Alpl-/-, aka Akp2-/-) mice closely phenocopy infantile HPP, including the rickets, vitamin B6-responsive seizures, improper dentin mineralization, and lack of acellular cementum. Here, we report that lack of TNAP in Alpl-/- mice also causes severe enamel defects, which are preventable by enzyme replacement with mineral-targeted TNAP (ENB-0040). Immunohistochemistry was used to map the spatiotemporal expression of TNAP in the tissues of the developing enamel organ of healthy mouse molars and incisors. We found strong, stage-specific expression of TNAP in ameloblasts. In the Alpl-/- mice, histological, mu CT, and scanning electron microscopy analysis showed reduced mineralization and disrupted organization of the rods and inter-rod structures in enamel of both the molars and incisors. All of these abnormalities were prevented in mice receiving from birth daily subcutaneous injections of mineral-targeting, human TNAP at 8.2?mg/kg/day for up to 44 days. These data reveal an important role for TNAP in enamel mineralization and demonstrate the efficacy of mineral-targeted TNAP to prevent enamel defects in HPP. (C) 2012 American Society for Bone and Mineral Research.

Morphological Alterations in Newly Born Dentate Gyrus Granule Cells That Emerge after Status Epilepticus Contribute to Make Them Less Excitable

Computer simulations of external current stimulations of dentate gyrus granule cells of rats with Status Epilepticus induced by pilocarpine and control rats were used to evaluate whether morphological differences alone between these cells have an impact on their electrophysiological behavior. The cell models were constructed using morphological information from tridimensional reconstructions with Neurolucida software. To evaluate the effect of morphology differences alone, ion channel conductances, densities and distributions over the dendritic trees of dentate gyrus granule cells were the same for all models. External simulated currents were injected in randomly chosen dendrites belonging to one of three different areas of dentate gyrus granule cell molecular layer: inner molecular layer, medial molecular layer and outer molecular layer. Somatic membrane potentials were recorded to determine firing frequencies and inter-spike intervals. The results show that morphologically altered granule cells from pilocarpine-induced epileptic rats are less excitable than control cells, especially when they are stimulated in the inner molecular layer, which is the target area for mossy fibers that sprout after pilocarpine-induced cell degeneration. This suggests that morphological alterations may act as a protective mechanism to allow dentate gyrus granule cells to cope with the increase of stimulation caused by mossy fiber sprouting.

The Effectiveness of a Voice Training Program for Telemarketers

Purpose. To use a randomized design to evaluate the effectiveness of voice training programs for telemarketers via multidimensional analysis. Methods. Forty-eight telemarketers were randomly assigned to two groups: voice training group (n = 14) who underwent training over an 8-week period and a nontraining control group (n = 34). Before and after training, recordings of the sustained vowel /epsilon/ and connected were collected for acoustic and perceptual analyses. Results. Based on pre- and posttraining comparisons, the voice training group presented with a significant reduction in percent jitter (P = 0.044). No other significant differences were observed, and inter-rater reliability varied from poor to fair. Conclusions. These findings suggest that voice training improved a single acoustic dimension, but do not change perceptual dimension of telemarketers' voices.

White paper sobre Open Source, Open Access, open Standards e o futuro da catalogação

The discussions on the future of cataloging has received increased attention in the last ten years, mainly due to the impact of rapid development of information and communication technologies in the same period, which has provided access to the Web anytime, anywhere. These discussions revolve around the need for a new bibliographic framework to meet the demand of this new reality in the digital environment, ie how libraries can process, store, deliver, share and integrate their collections (physical, digital or scanned), in current post-PC era? Faced with this question, Open Access, Open Source and Open Standards are three concepts that need to receive greater attention in the field of Library and Information Science, as it is believed to be fundamental elements for the change of paradigm of descriptive representation, currently based conceptually on physical item rather than intellectual work. This paper aims to raise and discuss such issues and instigate information professionals, especially librarians, to think, discuss and propose initiatives for such problems, contributing and sharing ideas and possible solutions, in multidisciplinary teams. At the end is suggested the effective creation of multidisciplinary and inter-institutional study groups on the future of cataloging and its impact on national collections, in order to contribute to the area of descriptive representation in national and international level

Comparative analysis of shell occupation by two southern populations of the hermit crab Loxopagurus loxochelis (Decapoda, Diogenidae)

The present study aimed to comparatively verify the relation between the hermit crabs and the shells they use in two populations of Loxopagurus loxochelis. Samples were collected monthly from July 2002 to June 2003, at Caraguatatuba and Ubatuba Bay, São Paulo, Brazil. The animals sampled had their sex identified, were weighed and measured; their shells were identified, measured and weighed, and their internal volume determined. To relate the hermit crab's characteristics and the shells' variables, principal component analysis (PCA) and a regression tree were used. According to the PCA analysis, the three gastropod shells most frequently used by L. loxochelis varied in size. The regression tree successfully explained the relationship between the hermit crab's characteristics and the internal volume of the inhabited shell. It can be inferred that the relationship between the morphometry of an individual hermit crab and its shell is not straightforward and it is impossible to explain only on the basis of direct correlations between the body's and the shell's attributes. Several factors (such as the morphometry and the availability of the shell, environmental conditions and inter- and intraspecific competition) interact and seem to be taken into consideration by the hermit crabs when they choose a shell, resulting in the diversified pattern of shell occupancy shown here and elsewhere.