Book Review: The Tree of Liberty: A Documentary History of Rebellion and Political Crime in America. Edited by Nicholas N. Kittrie and Eldon D. Wedlock, Jr.

Book review: The Tree of Liberty: A Documentary History of Rebellion and Political Crime in America. Edited by Nicholas N. Kittrie and Eldon D. Wedlock, Jr. Baltimore, Md.: Johns Hopkins University Press. 1986. Pp. 714. Reviewed by: Robert A. Rutland

Reviews

The following publications have been reviewed by the mentioned authors;Design and technology education exhibition  - reviewed by John EgglestonProject 2045 - The Design CouncilBeginning Teaching: Beginning Learning - reviewed by Bridget A. EganThe World of Chocolate - reviewed by Richard AgerSteel & Motorways Curriculum Pack - reviewed by Robert BowenDesign and Technology in Action Teacher's pack - reviewed by Robert Bowenthings: thinking things through with 7-11 year olds - reviewed by Marion RutlandD&T Challenges - reviewed by Tim FulfordSTEP Key Stage 3 Teacher's Handbook 2nd Ed. - reviewed by John HansonDesign and Technology Projects for Secondary Schools - reviewed by George Asquith

Targeted NGS gene panel identifies mutations in RSPH1 causing primary ciliary dyskinesia and a common mechanism for ciliary central pair agenesis due to radial spoke defects.

Primary ciliary dyskinesia (PCD) is an inherited chronic respiratory obstructive disease with randomized body laterality and infertility, resulting from cilia and sperm dysmotility. PCD is characterized by clinical variability and extensive genetic heterogeneity, associated with different cilia ultrastructural defects and mutations identified in >20 genes. Next generation sequencing (NGS) technologies therefore present a promising approach for genetic diagnosis which is not yet in routine use. We developed a targeted panel-based NGS pipeline to identify mutations by sequencing of selected candidate genes in 70 genetically undefined PCD patients. This detected loss-of-function RSPH1 mutations in four individuals with isolated central pair (CP) agenesis and normal body laterality, from two unrelated families. Ultrastructural analysis in RSPH1-mutated cilia revealed transposition of peripheral outer microtubules into the 'empty' CP space, accompanied by a distinctive intermittent loss of the central pair microtubules. We find that mutations in RSPH1, RSPH4A and RSPH9, which all encode homologs of components of the 'head' structure of ciliary radial spoke complexes identified in Chlamydomonas, cause clinical phenotypes that appear to be indistinguishable except at the gene level. By high-resolution immunofluorescence we identified a loss of RSPH4A and RSPH9 along with RSPH1 from RSPH1-mutated cilia, suggesting RSPH1 mutations may result in loss of the entire spoke head structure. CP loss is seen in up to 28% of PCD cases, in whom laterality determination specified by CP-less embryonic node cilia remains undisturbed. We propose this defect could arise from instability or agenesis of the ciliary central microtubules due to loss of their normal radial spoke head tethering

Probing Composition and Molecular Mobility in Thin Spherical Films Using Nuclear Magnetic Resonance Measurements of Diffusion

The composition and molecular mobility within thin spherical liquid films have been investigated using nuclear magnetic resonance (NMR) diffusion measurements. These films were formed either on the surface of pores inside a sponge at low saturation or by adsorbed water on the outside of urea prills during caking. Using pulsed field gradient (PFG) NMR experiments, the molecular mobility within these liquid films was determined through analysis of the conditional probability density for displacement (propagator). Molecular diffusion coefficients were determined for films in the sponge and prill systems by fitting the experimental propagators using a model for diffusion on an array of isotropically distributed infinite planes. By comparing these diffusion coefficients with bulk diffusion coefficients for a range of concentrations of urea solutions (2.1 M, 6.2 M and saturated), it was possible to optimize the PFG experimental parameters to enable accurate determination of molecular diffusion in these spherical liquid films. Determination of the diffusion coefficients for a range of urea solutions in the sponge enabled identification of the composition of the film that formed on the surface of the urea prills. Analysis of these data showed that the liquid layers are composed of saturated urea solution covering the surface of the prills, with an estimated layer thickness on the order of 10–5 m. The shape of the propagators indicated the adsorbed water was uniformly distributed over the surface of the urea prills, rather than primarily in the meniscus between particles, which agrees with dye visualization experiments on a pair of urea prills during caking. This work provides the first quantitative measurements of diffusion in thin spherical films, which is a key parameter for determining what controls the presence and rate of bonding between adjacent particle surfaces