A novel mutation of the CLCN1 gene associated with myotonia hereditaria in an Australian cattle dog

BACKGROUND: Heritable myotonia is a genetic muscle disorder characterized by slow relaxation of skeletal muscles. The main clinical signs are skeletal muscle stiffness, especially after vigorous contraction, and muscle hypertrophy. Muscle stiffness may be enhanced by inactivity, and often is relieved by exercise. Myotonia can be inherited in an autosomal dominant or recessive manner (Thomsen- or Becker-type myotonia, respectively). In mice, goats, Miniature Schnauzer dogs, and most affected humans, the disorder is caused by mutations in CLCN1, which encodes the skeletal muscle voltage-gated chloride channel, Cl1C-1. HYPOTHESIS: We hypothesized that an Australian Cattle Dog with generalized muscle stiffness and hypertrophy examined at the Ontario Veterinary College would have a mutation in the CLCN1 gene. ANIMALS: A pure-bred Australian Cattle Dog from Ontario, Canada, was used. METHODS: Based on clinical signs and electromyographic test results, a diagnosis of myotonia hereditaria was made, and a muscle biopsy was collected for genetic analysis. RESULTS: Sequence data obtained from the affected dog confirmed that it was homozygous for a single base insertion in the CLCN1 coding sequence. This mutation would result in a truncated ClC-1 protein being expressed, which, based on molecular evidence from other studies, would result in functionally compromised chloride conduction in the skeletal muscles of the animal. CONCLUSIONS AND CLINICAL IMPORTANCE: To the authors' knowledge, this report describes the Ist case of myotonia in an Australian Cattle Dog and represents the 1st non-Schnauzer canine myotonia to be genetically characterized. In addition, we developed a polymerase chain reaction-based genetic screen to detect heterozygotes with this mutation in the at-large Australian Cattle Dog population.     

Some Ideas on Reconstruction of Villages inside the City

In this paper, the historical reasons of formation of the village inside the city are analyzed. It is indicated that such situation for the villages inside the city are the result of joint action of both the villagers and the government. The foundation of villages inside the city laid on the simple compensation for farmers in confiscating the rural land and lack of intelligence aids. In this paper, the ideas and measures for their reconstruction are put forward based on consideration of the reasons of their formation and difficulties in the reconstruction.     

Ananlysis on the Liability Financing in Leverage Buyouts

This article briefly introduces the principle and the character of the leverage buyouts since the rate of finance lever and the risk is high in the LBO, this article gives a game-theory analysis on the financing process between the purchaser and the investor in LBO by using the ROSS model. It is expected that some beneficial proposal to either side can be put forward by parting the equilibrium.in the last, it poses that LBO can be a way of broadening the financing channel on the annexation between the enterprises in our country.     

Controlling Techniques of Channel Sharing in HFC Network

The techniques of sharing a channel in HFC are original with regard to those of Ethernet and Ring. In this paper, the analysis is stressed on the method of the upstream bandwidth allocation, the collision resolving, and the relationship among them, beginning with MAP management message. The MAP is a complex frame, in which the messages of ranging opportunity needed by the CM at the access beginning, bandwidth allocation contention, bandwidth allocation and collision detection are included. The access way for a user is explained after the frame format and functions of a MAP are illustrated. Finally, upstream bandwidth allocation, the cause of collision, algorithm and the process to solve the collision and so on are studied.     

Performance Analysis of Anti-CW Interference in MC-CDMA Systems

The multicarrier-code division multiple access (MC-CDMA),which is the combination of direct sequence CDMA (DS-CDMA) and orthogonal frequency division multiplexing (OFDM) techniques, offers various advantages over single-carrier CDMA system. Some of the advantages are that MC-CDMA systems possess can remove the intersymbol interference (ISI) and enhance narrow-band interference suppression capabilities. So the MC-CDMA technique becomes a focus in the field of mobile communication. The authors introduce the typical MC-CDMA system model, discuss the decision statisticalvariable in Rayleigh fading channel included the CW interference, and give the expressions of probability of error in uplink. An approach is proposed with Modified Equal Gain Combining (MEGC) to decrease the effect of CW interference. Theoretical analysis and numerical results show that the anti-CW interference performance of MC-CDMA system can be improved used the MEGC approach.     

滴灌灌水器迷宫流道数值模拟与结构优化设计

FEMLAB软件是COMSOL公司生产的一个专业有限元数值分析软件包,是基于偏微分方程的科学和工程问题进行建模和仿真计算的交互开发环境系统。利用FEMLEB软件对迷宫滴头进行三维的流道参数建模,在迷宫流道流态分析、压力流量关系、结构分析、流速分布、抗堵塞性能模拟上,进行参数逻辑化的选择和确定,建立参数化灌水器结构与性能参数关系模型,从而达到产品设计的目的。     

A comparative description of two farmer-managed irrigation systems in Nepal

The organization of two farmer-managed irrigation systems in the western hills of Nepal is described by examining the ways in which the activities of water allocation, water distribution, maintenance, and resource mobilization are performed. Due to the topography and environment, these two organizations are structured primarily to mobilize the large amount of labor required for maintenance of the intake and canal. Both organizations precisely define each member's water allocation. In one system, water is allocated in proportion to the area of an individual's land holding, while in the other water allocation is by purchased shares. These two cases were used to analyze the importance of the principle of water allocation for expansion of area irrigated and equity of access to irrigation. Evidence from the two systems shows that in this hill environment water allocation by purchased shares provides the individual incentive and an organizational mechanism for efficient development of irrigation resources. Expansion of area irrigated and equity of access to irrigation were found to be greater in the system which allocates water by purchased shares than where water was allocated in proportion to area irrigated.     

Passage of sediment through flumes and over weirs

This paper reports on laboratory research on the sediment passagecapability through long-throated flumes and broad-crested weirs withwhich the Froude number in the approach channel does not exceed 0.6over a distance of about 20 times the water depth upstream of thestructure. Design rules are given for any combination of flume or weirin combination with any channel.     

万亩农田节水灌溉综合开发区的规划与效果

通过对开发区农田生态系统的综合论证,提出研究目标、技术决策和规划原则,采用水利、农业相结合的节水技术体系,运用汇水、输水、调水、配水相结合的方式,联合调用地表、地下水资源,提高水的利用率,为中低产贫水区大面积推广综合节水灌溉提供可行的技术模式。     

Resource allocation tradeoffs in Manila’s peri-urban vegetable production systems: An application of multiple objective programming

This paper designed and developed a multi-objective programming (MOP) model to illustrate the dynamic relationship among technologies, productive activities, constraints and farmers’ objectives in the peri-urban vegetable production system and use the model as an economic tool in analysing probable consequences of a given action or innovation on the farm. The best compromise solution was generated using four analytical steps, as follows: single-objective optimization (to determine the ideal and anti-ideal values of the objective functions); constrained optimization (to generate the set of Pareto non-dominated solutions); cluster analysis (to trim down efficient set into smaller homogeneous groups); and compromise programming (to determine where the best compromise solution lies).