Genome stability, progressive kidney failure and aging

Two new studies report mutations in FAN1 and three other genome-stability genes that tie the DNA damage response to progressive kidney failure and the dysfunction of several other organs. These findings provide clues to the underlying causes of tissue decline and may add a series of genes to the growing list of genome maintenance factors that protect against premature aging.     

Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm

Loeys-Dietz syndrome (LDS) associates with a tissue signature for high transforming growth factor (TGF)-β signaling but is often caused by heterozygous mutations in genes encoding positive effectors of TGF-β signaling, including either subunit of the TGF-β receptor or SMAD3, thereby engendering controversy regarding the mechanism of disease. Here, we report heterozygous mutations or deletions in the gene encoding the TGF-β2 ligand for a phenotype within the LDS spectrum and show upregulation of TGF-β signaling in aortic tissue from affected individuals. Furthermore, haploinsufficient Tgfb2(+/-) mice have aortic root aneurysm and biochemical evidence of increased canonical and noncanonical TGF-β signaling. Mice that harbor both a mutant Marfan syndrome (MFS) allele (Fbn1(C1039G/+)) and Tgfb2 haploinsufficiency show increased TGF-β signaling and phenotypic worsening in association with normalization of TGF-β2 expression and high expression of TGF-β1. Taken together, these data support the hypothesis that compensatory autocrine and/or paracrine events contribute to the pathogenesis of TGF-β-mediated vasculopathies.     

Potassium and sodium transport in non-animal cells: the Trk/Ktr/HKT transporter family

Bacterial Trk and Ktr, fungal Trk and plant HKT form a family of membrane transporters permeable to K⁺ and/or Na⁺ and characterized by a common structure probably derived from an ancestral K⁺ channel subunit. This transporter family, specific of non-animal cells, displays a large diversity in terms of ionic permeability, affinity and energetic coupling (H⁺–K⁺ or Na⁺–K⁺ symport, K⁺ or Na⁺ uniport), which might reflect a high need for adaptation in organisms living in fluctuating or dilute environments. Trk/Ktr/HKT transporters are involved in diverse functions, from K⁺ or Na⁺ uptake to membrane potential control, adaptation to osmotic or salt stress, or Na⁺ recirculation from shoots to roots in plants. Structural analyses of bacterial Ktr point to multimeric structures physically interacting with regulatory subunits. Elucidation of Trk/Ktr/HKT protein structures along with characterization of mutated transporters could highlight functional and evolutionary relationships between ion channels and transporters displaying channel-like features.     

Protein constituents of the eggshell: eggshell-specific matrix proteins

In this article, we review the results of recent proteomic and genomic analyses of eggshell matrix proteins and draw attention to the impact of these data on current understanding of eggshell formation and function. Eggshell-specific matrix proteins from avian (ovocleidins and ovocalyxins) and non-avian (paleovaterin) shells are discussed. Two possible roles for eggshell-specific matrix proteins have been proposed; both reflect the protective function of the eggshell in avian reproduction: regulation of eggshell mineralization and antimicrobial defense. An emerging concept is the dual role (mineralization/antimicrobial protection) that certain eggshell matrix proteins can play.     

On a dynamic mixture GARCH model

This paper proposes a new mixture GARCH model with a dynamic mixture proportion. The mixture Gaussian distribution of the error can vary from time to time. The Bayesian Information Criterion and the EM algorithm are used to estimate the number of parameters as well as the model parameters and their standard errors. The new model is applied to the S&P500 Index and Hang Seng Index and compared with GARCH models with Gaussian error and Student's t error. The result shows that the IGARCH effect in these index returns could be the result of the mixture of one stationary volatility component with another non‐stationary volatility component. The VaR based on the new model performs better than traditional GARCH‐based VaRs, especially in unstable stock markets. Copyright © 2008 John Wiley & Sons, Ltd.     

自调式镦压挤胀复合液压机的研制

从力学原理、设计原理、整机结构、关键零部件的设计和工作程序，系统地介绍了自调式镦压挤胀复合液压机．由于压机设计了顶出缸对下活动横梁调节限位结构，回程拉杆对上镦压横梁的复位结构及镦压缸和气液储能器之间的连通协调结构，这不仅使该液压机结构紧凑，同时节省了上镦压横梁的回程液压缸、下活动横梁的镦压缸，并简化了上凸模与上镦压模分设的液压系统，而且解决了直齿圆柱齿轮在塑性成形过程中齿顶难以充满、齿根易出现微裂纹，以及成形压力过大和模具寿命过低的问题．     

Evolutionary genetics. Clonal inheritance of avian mitochondrial DNA.

We have taken a new approach to test the commonly accepted, but recently questioned, principle of clonal inheritance of vertebrate mitochondrial DNA (mtDNA) by relating its inheritance to a female-specific marker of nuclear DNA. Whereas this is impossible in organisms with male heterogamy (such as mammals), we show here that genealogies of mtDNA and the female-specific W chromosome of a bird species are completely concordant. Our results indicate that inheritance of mtDNA is free of detectable recombination effects over an evolutionary timescale.