Mutations in the gene encoding the basal body protein RPGRIP1L, a nephrocystin-4 interactor, cause Joubert syndrome

Protein-protein interaction analyses have uncovered a ciliary and basal body protein network that, when disrupted, can result in nephronophthisis (NPHP), Leber congenital amaurosis, Senior-L?ken syndrome (SLSN) or Joubert syndrome (JBTS). However, details of the molecular mechanisms underlying these disorders remain poorly understood. RPGRIP1-like protein (RPGRIP1L) is a homolog of RPGRIP1 (RPGR-interacting protein 1), a ciliary protein defective in Leber congenital amaurosis. We show that RPGRIP1L interacts with nephrocystin-4 and that mutations in the gene encoding nephrocystin-4 (NPHP4) that are known to cause SLSN disrupt this interaction. RPGRIP1L is ubiquitously expressed, and its protein product localizes to basal bodies. Therefore, we analyzed RPGRIP1L as a candidate gene for JBTS and identified loss-of-function mutations in three families with typical JBTS, including the characteristic mid-hindbrain malformation. This work identifies RPGRIP1L as a gene responsible for JBTS and establishes a central role for cilia and basal bodies in the pathophysiology of this disorder.     

ROLE OF α-PSEUDO-UNIVEX FUNCTIONS IN VECTOR VARIATIONAL-LIKE INEQUALITY PROBLEMS

In this paper, we introduce a new class of generalized convex function, namely, a-pseudounivex function, by combining the concepts of pseudo-univex and α-invex functions. Further, we establish some relationships between vector variational-like inequality problems and vector optimization problems under the assumptions of α-pseudo-univex functions. Results obtained in this paper present a refinement and improvement of previously known results.     

A New Discipline of Science-The Study of Open Complex Giant System and Its Methodology

This paper introduces the conception of open complex giant system and the methodology for dealing with the system, with stress on its profound significance in development of science and technology. The authors conclude that the reductionism underlying the exact science is not suitable to open complex giant system, and the only feasible alternative is the meta-synthetic engineering from the qualitative to the quantitative.     

Channel Mismatching in Sidelobe Canceler: Limitations and Remedies

After a brief recall of the Sidelobe Canceler (SLC) working principle, including the derivation of a general formula for the Cancellation Ratio (CR), the effects of channel mismatching are investigated. In particular, curves providing CRvalues as a function of amplitude and phase channel mismatching, radar bandwidth, and jammer direction of arrival (JDOA) are provided for the cases of one and two auxiliary antennas. Subsequently, a time -space processor for performance restoration is analyzed in detail. In addition to the above mentioned quantities, the attainable CR value is expressed as a function of the space-time processor parameters. The contribution of the paper is related to the derivation of a number of mathematical equations of CR for several cases of practical interest for the radar engineer. In addition, several curves are presented to assist design of SLC systems.     

未来十年的全球水源状况

1940年至1980年间,全球的用水量翻了一番,预计到2000年还要再翻一番。由于生活水平的差异,每人每天的生活用水量也相差甚大,为20～500升。全世界有4％的人均日用水量达300～400升(美国为580升),而全世界67％的人口(主要集中在非洲和亚洲)人均日用水量小于50升。时至今日,全球范围内,仍有65％的农村人口,35％的城市人口没有供水系统。尽管如此,在过去的10年里,又有13亿人喝上了卫生的水。但是,在现在52亿的世界人口中,仍有约12亿人喝不上卫生的水。     

Algorithm of graph isomorphism with threedimensional DNA graph structures

ResearchonDNAcomputingwasinitializedin1994 ,whenAdleman[1] proposedamethodofsolvingasmallinstanceoftheHamiltonianPathproblembyalaboratoryexperimentinvolvingDNAmolecules .Later,Lipton[2 ] demonstratedhowalargeclassofNP completeproblemscouldbesolvedbyencodingtheprobleminDNAmolecules .Inparticular ,LiptonshowedonefamousNP problem ,theso called“satisfiability”problem (SAT)andsubsequentlytheotherNP problemscouldbeencodedandsolvedusingmolecules .TheadvantagesofDNAcomputingareitsmassivepa…     

Mechanism and Defense on Malicious Code

With the explosive growth of network applications, the threat of the malicious code against network security becomes increasingly serious. In this paper we explore the mechanism of the malicious code by giving an attack model of the maliciouscode, and discuss the critical techniques of implementation and prevention against the malicious code. The remaining problems and emerging trends in this area are also addressed in the paper.     

行走机器人

本文简单地回顾了行走机器人的研究概况,较详细地描述了HILARE机器人,集中讨论了研制中的一些关键问题:环境感知、决策制定、执行控制以及HILARE计划中所试验的空间建模和路径寻找技术。最后讨论了行走机器人的发展方向及其在现实中的应用。