A high purity measurement of RbR_b at SLD

Precision measurement of Rb can provide important information about the Standard Model and beyond. SLD has developed a new method for measuring Rb with very high purity. This measurement has the lowest systematic error reported to date and future measurements using this method will likely have the lowest total uncertainty. This paper will be divided into the five sections: introduction, hardware, topological vertexing tag method, results and conclusions. The introduction will discuss the importance of Rb and the problems with other measurement techniques. The hardware section will give a brief description of the SLC/SLD system concentrating on its advantages over LEP. An outlook towards the future of SLD Rb measurements will be included in the conclusions

Behavioral and molecular exploration of the AR-CMT2A mouse model Lmna R298C/R298C

International audienceIn 2002, we identified LMNA as the first gene responsible for an autosomal recessive axonal form of Charcot-Marie-Tooth disease, AR-CMT2A. All patients were found to be homozygous for the same mutation in the LMNA gene, p.Arg298Cys. In order to investigate the physiopathological mechanisms underlying AR-CMT2A, we have generated a knock-in mouse model for the Lmna p.Arg298Cys mutation. We have explored these mice through an exhaustive series of behavioral tests and histopathological analyses, but were not able to find any peripheral nerve phenotype, even at 18 months of age. Interestingly at the molecular level, however, we detect a downregulation of the Lmna gene in all tissues tested from the homozygous knock-in mouse Lmna (R298C/R298C) (skeletal muscle, heart, peripheral nerve, spinal cord and cerebral trunk). Importantly, we further reveal a significant upregulation of Pmp22, specifically in the sciatic nerves of Lmna (R298C/R298C) mice. These results indicate that, despite the absence of a perceptible phenotype, abnormalities exist in the peripheral nerves of Lmna (R298C/R298C) mice that are absent from other tissues. Although the mechanisms leading to deregulation of Pmp22 in Lmna (R298C/R298C) mice are still unclear, our results support a relation between Lmna and Pmp22 and constitute a first step toward understanding AR-CMT2A physiopathology