Christian missions : their agents and their results (vol. 1) / by T.W.M. Marshall.

http://books.google.com/books?vid=ISBN0665456816&id=sipohllLjKQC&dq=protestant+missions&a_sbrr=1 View book via Google

Safety and efficacy of recombinant alpha(1)-antitrypsin therapy in cystic fibrosis.

Neutrophil elastase (NE) is thought to be the most important protease which damages the cystic fibrosis (CF) lung. Attempts have been made to suppress this activity using the plasma-derived inhibitor, alpha1-antitrypsin (AAT). In this pilot study, the safety and efficacy of inhaled recombinant human AAT (rAAT) as a treatment for CF were investigated. Thirty-nine patients participated in a prospective, double-blinded, randomized, placebo-controlled phase II trial to examine the effect of rAAT (500, 250, and 125 mg) on sputum NE activity. Sputum myeloperoxidase (MPO), interleukin-8, tumor necrosis factor receptors, sputum and plasma NE/AAT complexes, and safety parameters were also measured. Subjects were randomized to receive nebulized treatment once a day for 4 weeks, followed by 2–4 weeks with no study treatment, and then a 2-week rechallenge phase. Trends toward a reduction in NE activity were observed in patients treated with 500 mg and 250 mg of rAAT compared to placebo. Sputum NE/AAT complex and MPO levels were lower on rAAT compared to placebo. No major adverse events and, in particular, no allergic reactions to rAAT were observed. Although significant differences between rAAT and placebo for sputum NE activity were not observed, some improvements were found for secondary efficacy variables. This study demonstrated that nebulized rAAT is safe and well-tolerated, but has a limited effect on NE activity and other markers of inflammation. Pediatr Pulmonol.

Nestor-Guillermo Progeria Syndrome: a biochemical insight into Barrier-to-Autointegration Factor 1, alanine 12 threonine mutation

Background: Premature aging syndromes recapitulate many aspects of natural aging and provide an insight into this phenomenon at a molecular and cellular level. The progeria syndromes appear to cause rapid aging through disruption of normal nuclear structure. Recently, a coding mutation (c.34G > A [p.A12T]) in the Barrier to Autointegration Factor 1 (BANF1) gene was identified as the genetic basis of Nestor-Guillermo Progeria syndrome (NGPS). This mutation was described to cause instability in the BANF1 protein, causing a disruption of the nuclear envelope structure.

Results: Here we demonstrate that the BANF1 A12T protein is indeed correctly folded, stable and that the observed phenotype, is likely due to the disruption of the DNA binding surface of the A12T mutant. We demonstrate, using biochemical assays, that the BANF1 A12T protein is impaired in its ability to bind DNA while its interaction with nuclear envelope proteins is unperturbed. Consistent with this, we demonstrate that ectopic expression of the mutant protein induces the NGPS cellular phenotype, while the protein localizes normally to the nuclear envelope.

Conclusions: Our study clarifies the role of the A12T mutation in NGPS patients, which will be of importance for understanding the development of the disease.

Joseph Priestley: the man who drew time

The Joseph Priestley House Museum at Northumberland, Pennsylvania became interested in Priestley's pioneering timelines as a complement to his better known work in chemistry, electricity, biblical scholarship and political radicalism. Boyd Davis wrote this short article for the newsletter published by the Friends of the museum. The article concentrates on the connections between Priestley, his French contemporary Barbeu-Dubourg and Benjamin Franklin at the time of the struggle for American Independence, and Priestley's two key chronographic innovations: the use of drawn or printed lines to represent the duration of lives, and the associated use of dots to show when the dates of such lives are in doubt or dispute. Keywords: timeline, chronographics

Répertoire général pratique de notariat, de droit civil et fiscal et de formules d'actes : recueil périodique 1° de lois et décrets, avec commentaires ; 2° de jugements, arrêts, solutions, décisions, etc. ; 3° et de formules d'actes notariés : faisant suite au Traité-pratique et formulaire général du notariat / par A. Defrénois

Etat de collection : retirages de 1877-1878

Répertoire général pratique de notariat, de droit civil et fiscal et de formules d'actes : recueil périodique 1° de lois et décrets, avec commentaires ; 2° de jugements, arrêts, solutions, décisions, etc. ; 3° et de formules d'actes notariés : faisant suite au Traité-pratique et formulaire général du notariat / par A. Defrénois

1877 (T2,N1073)- (T2,N2171).

Lettre du Roy, envoyée à Mgr l'abbé de Sainct Germain Desprez, ou à son vicaire général en son absence, pour l'exhorter de faire chommer la feste de sainct Joseph dans l'estendüe de sa jurisdiction

[Acte royal. 1661-03-16. Paris]