A recurrent mutation in MED12 leading to R961W causes Opitz-Kaveggia syndrome

Opitz-Kaveggia syndrome (also known as FG syndrome) is an X-linked disorder characterized by mental retardation, relative macrocephaly, hypotonia and constipation. We report here that the original family for whom the condition is named and five other families have a recurrent mutation (2881C>T, leading to R961W) in MED12 (also called TRAP230 or HOPA), a gene located at Xq13 that functions as a thyroid receptor-associated protein in the Mediator complex.     

A Maturing of Systems Thinking? Evidence from Three Perspectives

This paper reviews trends in systems theory/thinking from the 1970s to the early 2000s. It proposes a maturation of the field based on certain conceptual and methodological advances that have sought to liberate systems thinking from earlier strictures. An edited dialogue among three prominent systems thinkers from different systems schools—Merrelyn Emery, Bob Flood, and Eric Wolstenholme—provides evidence. Similarities and differences are identified, complementarities among the schools are derived and analyzed, and trajectories for future research are indicated.     

STRATEGIC EXERCISE OF REAL OPTIONS：INVESTMENT DECISIONS IN TECHNOLOGICAL SYSTEMS

Viewing investment projects in new technologies as real options, this paper studies the effects ofendogenous competition and asymmetric information on the strategic exercise of real options. We firstdevelop a multi-period, game-theoretic model and show how competition leads to early exercise andaggressive investment behaviors and how competition erodes option values. We then relax the typicalfull-information assumption found in the literature and allow information asymmetry to exist acrossfirms. Our model shows, in contrast to the literature that payoff is independent of the ordering ofexercise, that the sequential exercise of real options may generate both informational and payoffexternalities. We also find some surpising but interesting results such as having more information isnot necessarily better.     

2010 BP Oil Spill and the Systemic Construct of the Gulf Coast Shrimp Supply Chain

On April 20, 2010, the blast on the rig Deepwater Horizon, and the ensuing disaster known as the BP oil spill, has disrupted the Gulf Coast Shrimp supply chain. Six elements of the systemic supply and demand side of that supply chain are identified and discussed. On the supply side are shrimpers, processors, wholesalers, retailers, and restaurants. On the demand side is the consumer. Qualitative investigative methods shed light upon the systemic practice within the gulf coast shrimp industry in the United States.     

Management Factors Influencing Location Selection Decisions of Independent Filmmakers: An Exploratory Case Study

This is an exploratory case study of a management issue that confronts filmmakers: how do they select locations for their films? The outcome of those decisions can have vast and immediate effects upon the profitability of a film. This is a sometimes shadowy, but significant industry with a major economic impact. For systems theory advocates, students, and explorers, this study demonstrates that investigative, immersive action research techniques can be used to explore what are otherwise hidden, somewhat cloistered business organizations and industry structures that are ordinarily closed to outsiders. The soft-system of this industry that is rooted upon trust, loyalty, alliances, relationships, and other non-financial bonds influence its decisions, and its institutional performance. While this research is focused upon a fragment of the overall film industry worldwide, and is thus limited in the universality of the propositions that subsequently emerge, this study can serve as a springboard for research into other soft systems, from which new knowledge of managerial perspectives can emerge.     

BOOK REVIEWS

Rethinking the Fifth Discipline: Learning Within the Unknowable by Robert Louis Flood. Sami Potatoes: Living with Reindeer and Perestroika by Michael Robinson and Karim-Aly Kassam. The Self Managing Organization: How Leading Companies Are Transforming the Work of Teams for Real Impact by Ronald E. Purser and Steven Cabana.     

The Third Track of the Open-Systems-Thinking School: An Application of Domain Theory to New Zealand Ports

The Open-Systems Thinking (OST) school identified with Emery and Trist is situated as an important but underutilized approach in systems thinking. The main features of OST are described, including four "tracks" along which OST has developed. The third track, which focuses on interorganizational domains, is considered in detail. It is then applied to a case concerning the complex social dynamics of and in port domains in New Zealand. This exercise enables us to illuminate some lacunae in previous representations of the port domain research, to assess OST's third track regarding its relevance for systemic practice, and to help to bring OST to the table with other systems approaches that are all vying for the attention of researchers, practicing managers, students, and consultants.     

SERVICE VALUE NETWORKS： INTO PRACTICE

Modeling a business to match its customer perceived （and customer desired） outcomes, remains an on-going task. This paper considers shows how a service value networks （SVNs） approach may be engaged to model, and deliver, understanding of the front-end business and its direct engagement with its immediate （or front-end） customers. These front-end customers may be either： （1） its off-line, in-store customers - engaging directly with the business sales staff （or representative）, or-（2） its on-line, virtual customers - engaging with the business via internet or remote access. In this situation, both the business, and the customer draw upon their respective viewpoints, and both sides influence the interaction. External to these business and customer influences are additional factors that capture the immediate and broader global effects - termed environmental effects. These three business-customer engagement areas of influence are captured by a SVN SEM approach.
Without a business-customer encounter of some kind the likelihood of a services business, and a prospecting customer, successfully engaging in an exchange process is reduced. This exchange may be a physical, and/or services exchange, and/or an information and/or ideas exchange. To the business, this encounter is, in effect, a trade, and as a result, the business targets acquiring an economic exchange that will ultimately deliver a net positive economic outcome. To the customer, external and internal information feeds, servicing, perceived value, and satisfaction, are key encounter drivers. SVNs offers a new way to understand the business-customer encounter, and to then utilize this acquired knowledge to either： （1） build a strategic management model, or （2） reengineer its business networks, and to then create a re-positioned, more customer-aligned business operation - one that is capable of delivering win - win, competitive business-customer solutions.     

The Children's Planning Initiative: Researching Decision-Making for Children

This paper recounts the story of how a number of workers involved in the care of children decided that they needed to do more than improve their skills and began instead to question some of the assumptions on which child care planning processes are built. The paper records a growing consciousness about the value of action research in developing alternative models of decision-making. It leads us from early questioning, through model creation, and on into testing the model in a new and complex organizational setting. While other voices are quoted, the paper recounts the story from the viewpoint of two actors, practitioner/manager and consultant.