Mutations in SEC63 cause autosomal dominant polycystic liver disease

Mutations in PRKCSH, encoding the beta-subunit of glucosidase II, an N-linked glycan-processing enzyme in the endoplasmic reticulum (ER), cause autosomal dominant polycystic liver disease. We found that mutations in SEC63, encoding a component of the protein translocation machinery in the ER, also cause this disease. These findings are suggestive of a role for cotranslational protein-processing pathways in maintaining epithelial luminal structure and implicate noncilial ER proteins in human polycystic disease.     

A laser frequency comb that enables radial velocity measurements with a precision of 1 cm s(-1)

Searches for extrasolar planets using the periodic Doppler shift of stellar spectral lines have recently achieved a precision of 60 cm s(-1) (ref. 1), which is sufficient to find a 5-Earth-mass planet in a Mercury-like orbit around a Sun-like star. To find a 1-Earth-mass planet in an Earth-like orbit, a precision of approximately 5 cm s(-1) is necessary. The combination of a laser frequency comb with a Fabry-Pérot filtering cavity has been suggested as a promising approach to achieve such Doppler shift resolution via improved spectrograph wavelength calibration, with recent encouraging results. Here we report the fabrication of such a filtered laser comb with up to 40-GHz (approximately 1-A) line spacing, generated from a 1-GHz repetition-rate source, without compromising long-term stability, reproducibility or spectral resolution. This wide-line-spacing comb, or 'astro-comb', is well matched to the resolving power of high-resolution astrophysical spectrographs. The astro-comb should allow a precision as high as 1 cm s(-1) in astronomical radial velocity measurements.     

The complete family of genes encoding G proteins of Caenorhabditis elegans

Caenorhabditis elegans is the first animal whose genomic sequence has been determined. One of the new possibilities in post-sequence genetics is the analysis of complete gene families at once. We studied the family of heterotrimeric G proteins. C. elegans has 20 Galpha, 2 Gbeta and 2 Ggamma genes. There is 1 homologue of each of the 4 mammalian classes of Galpha genes, G(i)/G(o)alpha, G(s)alpha , G(q)alpha and G12alpha, and there are 16 new alpha genes. Although the conserved Galpha subunits are expressed in many neurons and muscle cells, GFP fusions indicate that 14 new Galpha genes are expressed almost exclusively in a small subset of the chemosensory neurons of C. elegans. We generated loss-of-function alleles using target-selected gene inactivation. None of the amphid-expressed genes are essential for viability, and only four show any detectable phenotype (chemotaxis defects), suggesting extensive functional redundancy. On the basis of functional analysis, the 20 genes encoding Galpha proteins can be divided into two groups: those that encode subunits affecting muscle activity (homologues of G(i)/G(o)alpha, G(s)alpha and G(q)), and those (14 new genes) that encode proteins most likely involved in perception.     

Exponential Stability of Interval Time-Varying Dynamical System with Multidelay

1 .ＩＮＴＲＯＤＵＣＴＩＯＮＴｈｅｓｔａｂｉｌｉｔｙｏｆｔｈｅｉｎｔｅｒｖａｌｍａｔｒｉｘａｎｄｕｎｃｅｒｔａｉｎｓｙｓｔｅｍｗｉｔｈｔｉｍｅｄｅｌａｙｈａｓｂｅｅｎａｃｏｎｓｉｄｅｒｅｄｂｙｒｅｓｅａｒｃｈｅｒｓｉｎｒｅｆｅｒｅｎｃｅｓ [1～ 1 3 ].Ｔｈｅｓｔａｂｉｌｉｔｙｏｆｔｈｅｆｏｌｌｏｗｉｎｇｉｎｔｅｒｖａｌｄｙｎａｍｉｃａｌｓｙｓｔｅｍｗｉｔｈｓｉｎｇｌｅｔｉｍｅｄｅｌａｙ ｘ(ｔ) =Ｎ[Ｐ ,Ｑ]ｘ(ｔ) +Ｎ[Ｃ ,Ｄ]ｘ(ｔ-τ)ｈａｓｂｅｅｎｃｏｎｓｉｄｅｒｅｄｉｎｐｒｅｖｉｏｕｓｒｅｆｅｒｅｎ…     

An Efficient Constrained Model Predictive Control Algorithm Based on Approximate Computation

1 .ＩＮＴＲＯＤＵＣＴＩＯＮＭｏｓｔｓｙｓｔｅｍｓａｒｅｓｕｂｊｅｃｔｔｏｃｏｎｓｔｒａｉｎｔｓａｎｄｔｈｅｄｅｓｉｇｎｏｆｃｏｎｔｒｏｌｌｅｒｓｆｏｒｓｕｃｈｓｙｓｔｅｍｓｉｓａｖｅｒｙａｃｔｉｖｅａｒｅａｏｆｒｅ ｓｅａｒｃｈｎｏｗａｄａｙｓ.Ｉｔｉｓｗｅｌｌ ｋｎｏｗｎｔｈａｔｍｏｄｅｌｐｒｅｄｉｃｔｉｖｅｃｏｎｔｒｏｌ (ＭＰＣ) ,ａｌｓｏｃａｌｌｅｄｒｅｃｅｄｉｎｇｈｏｒｉｚｏｎｃｏｎｔｒｏｌ(ＲＨＣ) ,ｈａｓｂｅｅｎｗｉｄｅｌｙａｎｄｓｕｃｃｅｓｓｆｕｌｌｙａｐｐｌｉｅｄｉｎｐｒｏｃｅｓｓｉ…     

A Study on the Design for Object-Oriented Adaptive Decision-Making Teams

1 .ＩＮＴＲＯＤＵＣＴＩＯＮＩｎＣ3Ｉｓｙｓｔｅｍｓ,ｒｅｃｅｉｖｉｎｇ ,ｔｒａｎｓｍｉｓｓｉｏｎ ,ｐｒｏｃｅｓｓｉｎｇａｎｄｄｉｓｐｌａｙｏｆｉｎｆｏｒｍａｔｉｏｎａｒｅｄｅｖｅｌｏｐｅｄｃｏｎｃｅｒｎｉｎｇｔｈｅｄｅ ｃｉｓｉｏｎｍａｋｉｎｇｏｆｃｏｍｍａｎｄｅｒｓ.Ｔｈｅｒｅｆｏｒｅ,ｔｈｅｍａｊｏｒｔａｓｋｏｆａＣ3Ｉｓｙｓｔｅｍｉｓｆｏｃｕｓｅｄｏｎｄｅｃｉｓｉｏｎｍａｋｉｎｇ.Ｗｉｔｈｔｈｅｒａｐｉｄｄｅｖｅｌｏｐｍｅｎｔｏｆｃｏｍｍｕｎｉｃａｔｉｏｎａｎｄｎｅｔｗｏｒｋ ,ｔｅａｍｄｅｃｉｓ…     

Endogenous Stackelberg Leadership with Uncertain Information

1 ＩＮＴＲＯＤＵＣＴＩＯＮＩｎｒｅｃｅｎｔｙｅａｒｓ,ｍａｎｙｅｆｆｏｒｔｓｈａｖｅｂｅｅｎｍａｄｅｔｏｅｘｐｌｏｒｅｎｏｎ ｃｏｏｐｅｒａｔｉｖｅｇａｍｅｔｈｅｏｒｙｆｒｏｍａｄｅｃｉｓｉｏｎ -ｔｈｅｏｒｅｔｉｃｖｉｅｗｐｏｉｎｔ.Ｏｐｐｏｓｉｔｅｔｏｔｈｅｔｒａｄｉｔｉｏｎａｌｖｉｅｗｏｆｍｉｘｅｄｓｔｒａｔｅｇｉｅｓａｓｃｏｎｓｃｉｏｕｓｒａｎｄｏｍｉｚａｔｉｏｎｓ,ｐｌａｙｅｒｓｄｏｎｏｔｐｅｒ ｆｏｒｍｒａｎｄｏｍｉｚａｔｉｏｎ .Ｏｎｅｐｌａｙｅｒｃｈｏｏｓｅｓａｄｅｆｉｎｉｔｅａｃｔｉｏ…     

Study on the Reliability Evaluation of Qualitative Indices in Multi-Objective Decision

Qualitative indices in multi-objective decision can usually be evaluated and measured by mathematical methods or models, but the obtained results are sometimes inaccurate because of fuzziness of indices. To improve the accuracy and reliability of the evaluation results, set-value statistic principle is applied, and accordingly four evaluation methods are obtained. Meanwhile, these methods are compared briefly.