Autosomal dominant Alport syndrome linked to the type IV collage alpha 3 and alpha 4 genes (COL4A3 and COL4A4)

Alport syndrome is a hereditary nephritis that may lead to end-stage renal disease (ESRD) in young adult life and is often associated with sensorineural deafness and/or ocular abnormalities. The majority of families are X-linked due to mutations in the COL4A5 gene at Xq22. Autosomal forms of the disease are also recognized with recessive disease, having been shown to be due to mutations in the COL4A3 and COL4A4 genes on chromosome 2. Familial benign haematuria has also been mapped to this region in some families.

Predicting IQ of biologically "at risk" children from age 3 to school entry:sensitivity and specificity of the Stanford-Binet Intelligence Scale IV

Predictive validity of the Stanford-Binet Intelligence Scale Fourth Edition (S-B IV) from age 3 years to ages 4-5 years was evaluated with biologically "at risk" children without major sensory or motor impairments (n = 236). Using the standard scoring, children with full scale IQ <or = 84 on the Wechsler Preschool and Primary Scale of Intelligence at age 4-5 years were poorly identified (sensitivity 54%) from the composite S-B IV score at age 3. However, sensitivity improved greatly to 78% by including as a predictor the number of subtests the child was actually able to perform at age 3 years. Measures from the Home Screening Questionnaire and ratings of mother-child interaction further improved sensitivity to 83%. The standard method for calculating the composite score on the S-B IV excludes subtests with a raw score of 0, which overestimates cognitive functioning in young biologically high risk children. Accuracy of early identification was improved significantly by considering the number of subtests the child did not perform at age 3 years.

From non-porous crystalline to amorphous microporous metal(IV) bisphosphonates

Porous layered hybrid materials have been prepared by the reaction of organo-bisphosphonate ligands, 4-(4'-phosphonophenoxy)phenylphosphonic, 4,4'-biphenylenbisphosphonic and phenylphosphonic acids, with metal(IV) cations (Zr and Sn). Crystalline Zr(IV) and Sn(IV) layered bisphosphonates were also prepared, which were non-porous. The amorphous M(IV) bisphosphonates showed variable compositions and textural properties ranging from mainly mesoporous to highly microporous solids with BET surface areas varying from 300 to 480 m(2) g(-1), micropore volumes ranging 0.10-0.20 cm(3)/g, and narrow porous size distributions for some materials. N-2 isotherms suggest that Sn(IV) derivatives show a comparatively higher micropore contribution than the Zr(IV) analogous at least for the ether-bisphosphonate hybrids. Sn(IV) bisphosphonates exhibit high microporosities without the need of using harmful DMSO as solvent. If ether-bisphosphonic acid is partially replaced by less expensive phenylphosphonic ligand, porous products are also obtained. P-31 and F-17 MAS NMR and XPS data revealed the presence of hydrogen-phosphonate groups and small (F-, Cl- and OH-) anions, which act as spacer ligands within the inorganic layers, in these hybrid materials. The complexity of the inorganic layers is higher for the Sn(IV) bisphosphonates likely due to the larger amount of small bridging anions including fluorides. It is suggested that the presence of these small inorganic ligands may be a key factor influencing both, the interaction of the inorganic layer with the bisphosphonate groups, which bridge the inorganic layers, and the generation of internal voids within a given inorganic layer. Preliminary studies of gases adsorption (H-2 and NO) have been carried out for selected Sn(IV) bisphosphonates. The H-2 adsorption capacity at 77 K and 1 bar was low, 0.26 wt%, but the NO adsorption capacity at similar to 1 bar and 298 K was relatively high, 4.2 wt%. Moreover, the hysteresis in the NO isotherms is indicative of partial strong irreversible adsorption of NO. (C) 2008 Elsevier Inc. All rights reserved.

Papers in Italian Archaeology IV, Vol 4 Classical and Medieval.:The Cambridge Conference

Malone , C. and S. Stoddart. (eds.). 1985. Cambridge: British Archaeological Reports, Oxford.

Papers in Italian Prehistory IV - Vol 2 - Prehistory.:The Cambridge Conference

Malone , C. and S. Stoddart. (eds.). S244 1985. Cambridge: British Archaeological Reports, Oxford.

Papers in Italian Archaeology IV. Vol. 3. Patterns in Protohistory.:The Cambridge Conference

Malone, C.A.T. and S.K.F. Stoddart. (eds.). S245 1985. Cambridge: British Archaeological Reports, Oxford.

Papers in Italian Archaeology IV - Vol 1 Landscape in Italian Archaeology:The Cambridge Conference

Malone, C.A.T. and S.K.F. Stoddart. (eds.). IV. S243 1985. Cambridge: British Archaeological Reports.

Law and Society: Volume IV: Substantive Areas

The thriving and well-established field of Law and Society (also referred to as Sociolegal Studies) has diverse methodological influences; it draws on social-scientific and arts-based methods. The approach of scholars researching and teaching in the field often crosses disciplinary borders, but, broadly speaking, Law and Society scholarship goes behind formalism to investigate how and why law operates, or does not operate as intended, in society. By exploring law’s connections with broader social and political forces—both domestic and international—scholars gain valuable perspectives on ideology, culture, identity, and social life. Law and Society scholarship considers both the law in contexts, as well as contexts in law.
Law and Society flourishes today, perhaps as never before. Academic thinkers toil both on the mundane and the local, as well as the global, making major advances in the ways in which we think both about law and society. Especially over the last four decades, scholarly output has rapidly burgeoned, and this new title from Routledge’s acclaimed Critical Concepts in Law series answers the need for an authoritative reference collection to help users make sense of the daunting quantity of serious research and thinking.
Edited by the leading scholars in the field, Law and Society brings together in four volumes the vital classic and contemporary contributions. Volume I is dedicated to historical antecedents and precursors. The second volume covers methodologies and crucial themes. The third volume assembles key works on legal processes and professional groups, while the final volume of the collection focuses on substantive areas. Together, the volumes provide a one-stop ‘mini library’ enabling all interested researchers, teachers, and students to explore the origins of this thriving sub discipline, and to gain a thorough understanding of where it is today.

Synthesizable high performance adaptive equaliser and viterbi decoder for the class-IV PRML channel

The design and VLSI implementation of two key components of the class-IV partial response maximum likelihood channel (PR-IV) the adaptive filter and the Viterbi decoder are described. These blocks are implemented using parameterised VHDL modules, from a library of common digital signal processing (DSP) and arithmetic functions. Design studies, based on 0.6 micron 3.3V standard cell processes, indicate that worst case sampling rates of 49 mega-samples per second are achievable for this system, with proportionally high sampling rates for full custom designs and smaller dimension processes. Significant increases in the sampling rate, from 49 MHz to approximately 180 MHz, can be achieved by operating four filter modules in parallel, and this implementation has 50% lower power consumption than a pipelined filter operating at the same speed.