bluuebunny/crossref_metadata_2025 huggingface dataset:
Stars on the Screen, Strangers on the Streets
Authors: Hall Sara
Published: November 2007

Jans B. Wager, Dangerous Dames. Women and Representation in the Weimar Street Film and Film Noir (Athens: Ohio University Press, 1999)

Katharina von Ankum, ed., Women in the Metropolis: Gender and Modernity in Weimar Culture (Berkeley: University of California Press, 1997)
Wer Spinnt?
Authors: Feldman Gerald D.
Published: November 2007

Christopher Simpson, ed., War Crimes of the Deutsche Bank and the Dresdner Bank. Office of Military Government (U.S.) Reports (New York and London: Holmes & Meier, 2001)
The GDR: Internal and External Constraints
Authors: Kopstein Jeffrey
Published: November 2007

Gareth Pritchard, The Making of the GDR: From Anti-Fascism to Stalinism, 1945-1953 (Manchester and New York: Manchester University Press, 2000)

M.E. Sarotte, Dealing with the Devil: East Germany and Ostpolitik, 1969-1973 (Chapel Hill and London: The University of North Carolina Press, 2001)
Clash Over Corporate Accountability and the Holocaust
Authors: Simpson Christopher
Published: November 2007

The most deceitful aspect of Gerald Feldman’s commentary on my

book is his tacit claim that he is engaged in something other than

character assassination. As in other academic jihads he has pursued in

the past, Feldman’s most effective weapon has been his capacity for

ad hominem attack. Straightforward debate concerning disputed historical

evidence is considerably further down his list.
On Ethnic Essence and the Notion of German Victimization: Martin Walser and Asta Scheib’s Armer Nanosh and the Jew within the Gypsy
Authors: Margalit Gilad
Published: November 2007

This article discusses a screenplay of the television thriller Armer

Nanosh (Poor Nanosh), written in 19891 by the famous German

author Martin Walser and Asta Scheib.2 The screenplay deals with

the relations between Germans and Germany’s Sinti, or Gypsy, population

in the shadow of Auschwitz,3 a subject that has hardly been

touched upon by postwar German authors and dramatists.
Parliamentary Control and Foreign Policy in Germany: The Bundestag’s Use of Formal, Instrumentalities in Overseeing the Administration’s Foreign Policy
Authors: Anderson James Ryan
Published: November 2007

In a little more than a decade, Germany’s role in international affairs—

particularly from a military perspective—has radically changed. Whereas

German participation during the Persian Gulf War of 1991 was

basically limited to providing financial support to the international

coalition led by the United States, by the end of 2001, German soldiers

were operating under combat conditions in the United Nations peacekeeping

mission to Afghanistan. During (and even before) this transition,

little attention has been devoted to the German Bundestag’s

constitutional role as overseer of executive foreign affairs activities.
Immigration and German Cities: Exploring National Policies and Local Outcomes
Authors: Heisler Barbara Schmitter
Published: November 2007

The presence of sizable foreign-born populations (which include citizens

and non-citizens) in advanced industrial societies is the result of

national policies on immigration, refugees, and labor migration. The

consequences of these policies, however, are most visible at the local

level, where newcomers work, settle, and raise their families. While

not all immigrants live in cities, they have been particularly concentrated

in urban environments. Thus, it is hardly surprising that many

of the socioeconomic, cultural, and political issues and problems

associated with immigration and the process of immigrant settlement

are played out and magnified in cities.
In Search of a Future: Leipzig Since the Wende
Authors: Kolinsky Eva
Published: November 2007

In the political and economic history of Germany, Leipzig already

held a special place long before unification. Since the middle ages, it

has hosted one of the most important trade fairs in Europe. When

industrialization turned Germany in the late nineteenth century into

a leading European power, outpacing France and closely rivaling

Britain, Leipzig added to its established and internationally acclaimed

fur and book trade a mighty industrial sector in lignite-based chemicals

and vehicle production. At the turn of the century, Leipzig was

one of the largest and most affluent cities of Germany and indeed

Europe. A rich stock of Gründerzeit houses remains to testify to this

illustrious past.
The New Berlin
Authors: Strom Elizabeth , Mayer Margit
Published: November 2007

National and world events shape all cities, but in Berlin they have a

physical presence. For Berliners, the Cold War was tangible, manifested

as a wall and death strip guarded by armed soldiers and attack

dogs. Today that wall is gone and, if national power brokers and the

real estate development community have their way, Berlin will soon

be a “normal” European city and German capital. Not only will the

ghosts of the Nazi past be exorcised, but any tangible inheritance of

the postwar period—in East Berlin the legacies of state socialism, in

West Berlin the strange fruits of a subsidized economy—will disappear.
Integration and Political Representation in a Multicultural City: The Case of Frankfurt am Main
Authors: Klopp Brett
Published: November 2007

Cities have long been the destination of those on the move. Migration

and especially immigration always raise issues of inclusion and

exclusion, of rights and obligations, and of the meaning of membership

and citizenship. The particular form and content of these

debates vary, just as host countries, national and local governments,

and immigrant populations vary. Over the past few decades, patterns

of immigration have begun to shift away from classical immigration

countries (the United States, Canada, Australia) toward the democracies

of the European Union. “In this troubled world, Western

Europe has in fact, become a fragile island of prosperity, peace,

democracy, culture, science, welfare and civil rights,” according to

urban sociologist, Manuel Castells. “However, the selfish reflex of

trying to preserve this heaven by erecting walls against the rest of

the world may undermine the very fundamentals of European culture

and democratic civilization, since the exclusion of the other is

not separable from the suppression of civil liberties and a mobilization

against alien cultures.”
Capitalist Futures and Socialist Legacies: Urban Development in East Germany Since 1990
Authors: Häußermann Hartmut
Published: November 2007

Since unification, the political, economic, and institutional structures

in the new federal states have been patterned in accordance with the

West German model. This is due in part to the extension of the

Western legal framework to the eastern Länder. The fact that the

political and economic actors of the once-socialist country are now

subject to the institutional conditions of the West encourages convergence

towards the western model. But questions have been raised as

to whether the cities in the new federal states are also adapting

rapidly to the western model of urban development. Their layout

and architecture resulted, after all, from the investment decisions

made by several generations and cannot be shifted or transformed as

rapidly as legal or institutional frameworks.
A Celebration of Democracy: The 1998 Bundestag Election
Authors: Dalton Russell J.
Published: November 2007

Free elections are celebrations of the democratic process, and the

Germans celebrated in an unprecedented way on September 27,

1998. After sixteen years of Christian Democratic rule, the public

used its democratic power to change the government. Indeed, for the

first time in the history of the Federal Republic, voters rejected a sitting

chancellor and chose a new government through the ballot box.
Representing German Victimhood and Guilt: The Neue Wache and Unified German Memory
Authors: Kattago Siobhan
Published: November 2007

Commemorating National Socialism and Communism from the perspective

of 1989 often results in an uneasy conflation of German

guilt and victimhood. When the events of 1933-1989 are presented

as one long authoritarian period, war and tyranny can easily be construed

as external forces that simply befell the German nation.

While memories of national guilt are divisive, memories of victimhood

unify and simplify an otherwise ambiguous past. The 1995

restoration of Berlin’s Neue Wache is emblematic of this conflation

of guilt and victimhood. As the central German memorial to all victims

of war and tyranny, the Neue Wache neither distinguishes

between dictatorships, nor between perpetrator and victim.
Explaining Far-Right Electoral Successes in Germany: The Politicization of Immigration-Related Issues
Authors: Karapin Roger
Published: November 2007

Most explanations that have been advanced regarding the recent

successes of far-right parties in Western Europe suggest that these

parties should have also done well in Germany. With a high percapita

income and a strong export-oriented economy, Germany has

experienced large-scale immigration, a shift toward postindustrial

occupations, economic restructuring, unemployment, and social

marginalization of the poorest strata. These socioeconomic developments

have been accompanied by political responses which

should also benefit the far right: political parties have lost credibility, non-voting has increased, and ecological parties have become

established and have spurred environmental, feminist, and proimmigrant

policies.
Context and Consequence: The Impact of the New Radical Right on the Political Process in France and Germany
Authors: Minkenberg Michael
Published: November 2007

International comparisons of new radical right-wing parties usually

focus on differences in electoral fortunes, party organizations, and

leadership styles and conclude that Germany stands out as a special

case of successful marginalization of the new radical right. Explanations

for this German anomaly point at the combined effects of German

history and institutional arrangements of the Federal Republic

of Germany, of ideological dilemmas and strategic failures of the

various parties of the new radical right, and the efforts of the established

political parties to prevent the rise of new parties to the right

of them. By implication, this means that, whereas in countries like

France or Austria the new radical right plays a significant role in politics

to the point of changing the political systems themselves, the

German counterpart has a negligible impact and has little or no

effects on politics and polity.
Problematic Normalization: Eastern German Workers Eight Years after Unification
Authors: Segert Astrid
Published: November 2007

How can one best investigate the mental attitudes and patterns of

behavior of eastern Germans eight years after political unification?

Since 1990, the method dominating this discussion has been based

on measuring the degree to which easterners have “caught up” with

the supposedly more modern western Germans. However, empirical

studies and surveys have shown that this model is an ineffective, even

inappropriate means of describing how unification has impacted the

lives of eastern Germans. In this article, I argue that a more appropriate

approach is to consider the enduring differences in the opportunity

structures among eastern and western Germans, as well as the

differences in their respective behavioral patterns. In this context,

“opportunity structure” refers to the opportunities provided and limitations

imposed by social structures. For the analysis of opportunity

structures, I focus on what I call “contradictory adaptation” and

“problematic normalization.” My analysis of behavioral patterns

emphasizes the logic internal to the subjects themselves (Eigenlogik).

This internal logic differs significantly from outsiders’ interpretations

of easterners’ behavior, as the following example illustrates.
Putting Humpty-Dumpty Back Together Again: Communism’s Collapse and the Reconstruction of the East German Ex-Communist Party
Authors: Ziblatt Daniel F.
Published: November 2007

The collapse of communism did not follow any single path in east

central Europe. In Hungary and Poland, the transition was marked

by early negotiations between opposition elites and the ruling Communist

party. In East Germany and Czechoslovakia, the regimes fell

victim to a sudden and quick implosion. In Romania and Bulgaria,

internal coups replaced the ruling communist elite with other members

of the nomenklatura. The transitions away from communist rule

diverged from each other in timing, manner, and degree.
The Politics of Memorial Representation: The Controversy Over the German Resistance Museum in 1994
Authors: Case J. David
Published: November 2007

The study of historical memory in its various forms is a burgeoning

area of inquiry among historians. The debate over public, official,

government-supported memory and private individual memories

reveals a complex dynamic among myth, memory, and history,

which as Michel Foucault and others have argued, is simply the dominant

form of memory in a society at a given time.1 Some of the most

revealing instances of the intersection between public and private

memory are commemorations and memorial sites where personal

memories are created and sustained within the context of the official

representation of the event and those involved. The constant need to

locate memories within a larger social frame of reference ensures

that supporters of different memories of the same event will directly

and forcefully link images from the present with their memories of

the past, no matter how incongruous these images may appear.
Immigration and Citizenship in Germany
Authors: Münz Rainer , Ulrich Ralf
Published: November 2007

In Germany, as in many other European democracies, immigration

and citizenship are contested and contentious issues. In the German

case it was both the magnitude of postwar and recent immigration as

well as its interference with questions of identity that created political

and social conflict. As a result of World War II, the coexistence

of two German states, and the persistence of ethnic German minorities

in central and eastern Europe, (West) Germany’s migration and

naturalization policy was inclusive toward expellees, GDR citizens,

and co-ethnics. At the same time, the Federal Republic of Germany,

despite the recruitment of several million foreign labor migrants

and—until 1992—a relatively liberal asylum practice, did not develop

similar mechanisms and policies of absorption and integration of its

legal foreign residents.
Managing Decentralization: The Strategy of Institutional Differentiation in German Industrial Relations
Authors: Schroeder Wolfgang , Weinert Rainer
Published: November 2007

The approach of the new millennium appears to signal the demise

of traditional models of social organization. The political core of

this process of change—the restructuring of the welfare state—and

the related crisis of the industrywide collective bargaining agreement

have been subjects of much debate. For some years now in

specialist literature, this debate has been conducted between the

proponents of a neo-liberal (minimally regulated) welfare state and

the supporters of a social democratic model (highly regulated). The

alternatives are variously expressed as “exit vs. voice,” “comparative

austerity vs. progressive competitiveness,” or “deregulation vs.

cooperative re-regulation.”
Mandarins in the Public Sphere: Vergangenheitsbewältigung and the Paradigm of Social History in the Federal Republic of Germany
Authors: Kansteiner Wulf
Published: November 2007

Since the 1960s, Germany’s historical culture has continually reprocessed

the Nazi past and later the Holocaust for the purposes of education,

remembrance, and entertainment. The objective of this process,

Vergangenheitsbewältigung, is the self-centered and self-designed

therapeutic treatment of the descendants of the perpetrators and

bystanders of Nazism. It seems that Germans, who were better fascists

than other Europeans, are also determined to excel at the task of

working through Nazism and the World War II era. Therefore,

attempts at mastering the past have given rise to hectic cultural activity

as the field of contemporary history illustrates: “[I]ncessantly the

German business for contemporary history generates fast-food products.

It is based on a perpetual mobile of commissions, projects and

mini-grants, temporary employment and welfare-to-work subsidies,

conferences and lecture series—a perpetual mobile of pedagogical historiography

and history obsessed pedagogy.”
Academic and Public Discourses on the Holocaust: The Goldhagen Debate in Germany
Authors: Herbert Ulrich
Published: November 2007

Over two years after the appearance of Hitler’s Willing Executioners,

very little can be heard about the so-called Goldhagen Debate in

Germany: no more scholarly reviews, at most a few echoes here and

there. Over two hundred thousand copies of the book were sold,

and it was certainly read almost as many times. But it does not

appear in the syllabi of university courses on the Holocaust, except

perhaps in those that cover historiographical debates. In the German

edition of Saul Friedländer’s new book, Nazi Germany and the Jews,

Daniel Goldhagen does not rate a mention, except for a three line

footnote on page 420 in which his theory is described as “unconvincing

on the basis of the materials presented as part of the study.”2

Goldhagen’s book, one can confidently predict, will not play a role

in future Holocaust research.
Memory and the Cityscape: The German Architectural Debate about Postmodernism
Authors: James-Chakraborty Kathleen
Published: November 2007

Few tools of Nazi propaganda were as potent or as permanent as

architecture. At the instigation of Hitler, who had once aspired to be

an architect, the Nazi regime placed unusual importance on the

design of environments—whether cities, buildings, parade grounds, or

highways—that would glorify the Third Reich and express its dynamic

relationship to both the past and the future. Architecture and urban

design were integral to the way the regime presented itself at home

and abroad. Newsreels supplemented direct personal experience of

monumental buildings. Designed to last a thousand years, these edifices

appeared to offer concrete testimony of the regime’s enduring

character. A more subtle integration of modern functions and vernacular

forms, especially in suburban housing, suggested that technological

progress could coexist with an “organic” national community

rooted in a quasi-sacred understanding of the landscape.
The Dilemmas of Commemoration
Authors: Hoffmann Christhard
Published: November 2007

A few weeks after the founding of the Federal Republic of Germany

in May 1949, American High Commissioner John McCloy addressed

an assembly of representatives from the West German Jewish community.

In a much-discussed speech, he emphasized the central

importance of public recollection of the crimes of the Third Reich for

the political culture of the young republic. In particular, said McCloy,

the relationship of West Germany towards the Jews would be “one of

the real touchstones and the test of Germany’s progress toward the

light. The moment that Germany has forgotten the Buchenwalds and

Auschwitzes, that was the point at which everyone could begin to

despair of any progress in Germany.”
Legacies of Divided Memory for German Debates about the Holocaust in the 1990s
Authors: Herf Jeffrey
Published: November 2007

The legacies of almost a half-century of divided memory continue to

influence commemoration of the Holocaust in unified Germany.

Because these practices were decisively shaped by the multiple

restorations of past political traditions in the early postwar period, I

will comment on the commemorations of the first two postwar

decades in East and West Germany and conclude with brief remarks

about how past legacies influence recent practices. I will examine

the significance of the Holocaust in these events compared to the

attention given to the suffering of Nazi Germany’s non-Jewish victims.

I will also consider the extent to which distinctions were made

among the various victims of Nazi Germany, the kind of hierarchies

that were established among them, and the use of commemoration

for political purposes.
National Identity and Recovering Memories in Contemporary Germany: The Reception of Victor Klemperer’s Diaries
Authors: Richards Earl Jeffrey
Published: November 2007

The overwhelming critical response in Germany to the publication of

Victor Klemperer’s journals, particularly those spanning the years

from 1933 to 1945, has been a veritable sensation. Hundreds of

reviews, mostly appreciations, have appeared. Klemperer’s journals

have also turned into big business. On October 12, 1999, the German

television channel ARD began broadcasting a thirteen-episode series

on the diaries in the most expensive, made-for-television program of

its kind in Germany. Additionally, the English-language rights to the

journals were sold to Random House for a record $550,000, more

than has ever been paid for translation rights of any German book in

history. The selling of Klemperer’s journals may have led to a distorted

evaluation of their author’s position and importance.
German Reunification from Three Angles
Authors: Livingston Robert Gerald
Published: November 2007

Robert L. Hutchings, American Diplomacy and the End of the Cold War: An Insider’s Account of U.S. Policy in Europe, 1989-1992 (Washington, D.C. and Baltimore: The Woodrow Wilson Center Press and The Johns Hopkins University Press, 1997)

Charles S. Maier, Dissolution: The Crisis of Communism and The End of East Germany (Princeton, New Jersey, Princeton University Press, 1997)

Peter E. Quint, The Imperfect Union: Constitutional Structures of German Unification (Princeton, New Jersey, Princeton University Press, 1997)
The Forty-Fivers: A Generation Between Fascism and Democracy
Authors: Moses A.D.
Published: November 2007

In 1999, Germans celebrate the fiftieth anniversary of the founding of the Federal Republic. Unlike the fiftieth anniversary of other events in the recent national past—the Nazi seizure of power in 1933, the anti-Jewish pogrom of November 1938, and the unconditional surrender in 1945—this is not an awkward occasion for the country’s elites. On the contrary, the Federal Republic is indisputably Germany’s most successful state, and its record of stability and prosperity compares favorably with that of two prominent neighbors, France and Italy. This anniversary gives us pause to pose the basic questions about West Germany. How was it possible to construct an enduring democracy for a population that, exceptions notwithstanding, had enthusiastically supported Hitler and waged world war to the bitter end?
German Foreign Policy in a Strategic Triangle: Bonn–Paris–Washington
Authors: Haftendorn Helga
Published: November 2007

German foreign policy operates in a strategic triangle, the corner points of which are Bonn, Paris, and Washington. This constellation dates to the end of World War II. Since that time, German foreign policy has been influenced by this strategic triangle, which provides for

political opportunities as well as for significant risks. It relies on the interdependence of German-American, German-French, and French-American relations.
Joint angle, moment and power compensations in dogs with fragmented medial coronoid process
Authors: Dobney J. A. , Owen M. R. , Colborne G. R. , Burton N. J.
Published: November 2007

Fragmented medial coronoid process (FMCP) is the most common cause of forelimb lameness in juvenile medium and large breed dogs; however methods of assessing the disruption to their gait remain subjective. The purpose of this study was to objectively quantify the mechanical disruptions to gait in dogs with arthroscopically confirmed unilateral FMCP. Seven dogs underwent full inverse dynamic analysis at the time of diagnosis. Kinematic and force data were collected from both forelimbs at trot. Stance phase joint angles, net joint moments and net joint powers were calculated using custom software. There were gross differences in kinetic and kinematic patterns between FMCP affected and compensating forelimbs. Stance time was 0.24 sec on the lame side and 0.26 sec on the compensating side. The shoulder and the elbow were more flexed at ground contact, and elbow, carpal and MCP joints had smaller ranges of motion on the lame side. Net joint moments were significantly reduced (P<0.05) in the elbow, carpal and MCP joints of the FMCP affected limb. Net joint powers were likewise significantly smaller (P<0.05). However, the overall moment and power patterns persisted. Total limb support moment was significantly smaller on the affected side (P<0.05). Total limb power was significantly reduced on the affected side (P<0.05) being most affected in its propulsive phase in the second half of stance. Inverse dynamic analysis of this clinical condition is an objective means by which to assess the mechanical disruption to gait.
Two cases of surgically treated feline patellar fractures
Authors: Scotti S. , Carozzo C. , Bouvy B. , Genevois J.-P. , Guillaumot P.
Published: November 2007

A transverse patellar fracture in a six-month-old cat was successfully treated by figure-of-eight dorsal wiring of the patella. A longitudinal patellar fracture with luxation of a large medial fragment in a 2.5-year-old cat was treated by lateral marginal patellectomy with a positive outcome. While adding material to the few veterinary reports in that species, in this brief communication, the authors discuss the aetiology, diagnosis, and the treatment of the presented cases with regards to findings in previously published feline cases.
Determination of Cholinesterase in Blood and Tissue
Authors: Wilson Barry W. , Henderson John D.
Published: November 2007

The widespread use of organophosphate and organocarbamate pesticides and the dangers of related chemical warfare agents dramatize the importance of rapid, accurate, and sensitive assays for blood and tissue cholinesterases (ChEs), important targets for neurotoxic chemicals. Two ChE enzymes used as biomarkers of exposure are the specific acetylcholinesterases (AChE, EC 3.1.1.7) and the nonspecific plasma cholinesterases (BChE, EC 3.1.1.8). This unit contains two protocols for measuring ChE activity: (1) a colorimetric kinetic method and (2) a radiometric endpoint assay and selective inhibitors that are used to distinguish between the two classes of enzymes. Curr. Protoc. Toxicol. 34:12.13.1‐12.13.16. © 2007 by John Wiley & Sons, Inc.
Comparative Protein Structure Modeling Using MODELLER
Authors: Eswar Narayanan , Webb Ben , Marti‐Renom Marc A. , Madhusudhan M.S. , Eramian David , Shen Min‐yi , Pieper Ursula , Sali Andrej
Published: November 2007

Functional characterization of a protein sequence is a common goal in biology, and is usually facilitated by having an accurate three‐dimensional (3‐D) structure of the studied protein. In the absence of an experimentally determined structure, comparative or homology modeling can sometimes provide a useful 3‐D model for a protein that is related to at least one known protein structure. Comparative modeling predicts the 3‐D structure of a given protein sequence (target) based primarily on its alignment to one or more proteins of known structure (templates). The prediction process consists of fold assignment, target‐template alignment, model building, and model evaluation. This unit describes how to calculate comparative models using the program MODELLER and discusses all four steps of comparative modeling, frequently observed errors, and some applications. Modeling lactate dehydrogenase from Trichomonas vaginalis (TvLDH) is described as an example. The download and installation of the MODELLER software is also described. Curr. Protoc. Protein Sci. 50:2.9.1‐2.9.31. © 2007 by John Wiley & Sons, Inc.
Protein Structure Analysis Online
Authors: Miteva Maria A. , Alexov Emil , Villoutreix Bruno O.
Published: November 2007

Computational biology/chemistry tools are used in most areas of life/health science research. These methods are continually being developed and their use can present difficulties for both experienced and novice investigators. To facilitate the use of these applications, many packages have been implemented online during these last 5 years. This unit focuses on online computational methods with a special emphasis on structural refinement/atomic simulations, protein electrostatic calculations, searches for functional sites, searches for druggable pockets, protein docking and small molecule docking, and prediction of potential impact of amino acid variations on the structure and function of the protein molecules. Curr. Protoc. Protein Sci. 50:2.13.1‐2.13.23. © 2007 by John Wiley & Sons, Inc.
Animal Models of Airway Sensitization
Authors: Pichavant Muriel , Goya Sho , Hamelmann Eckard , Gelfand Erwin W. , Umetsu Dale T.
Published: November 2007

Asthma is a complex phenotype that involves multiple mechanisms, including adaptive and innate immunity as well as physiological and mechanical changes in the airways. A cardinal feature of asthma is airway hyperreactivity (AHR), a multifaceted reaction that can only be assessed in vivo. Mouse models of asthma replicate many of the features of human asthma, including AHR, which can be assessed using standard protocols. Examination of AHR in mice has provided important information about human asthma, primarily because the immunology of allergy is easily studied in mice, especially with the availability of reagents including genetically modified mice. In this unit we discuss the induction and measurement of AHR and the two most common methodologies: noninvasive measurement using a whole‐body plethysmograph (WBP) and invasive measurement of lung resistance and dynamic compliance. Curr. Protoc. Immunol. 79:15.18.1‐15.18.19. © 2007 by John Wiley & Sons, Inc.
Measuring Protein‐Protein Interactions by Equilibrium Sedimentation
Authors: Balbo Andrea , Brown Patrick H. , Braswell Emory H. , Schuck Peter
Published: November 2007

This unit describes basic principles and practice of sedimentation equilibrium analytical ultracentrifugation for the study of reversible protein interactions, such as the characterization of self‐association, heterogeneous association, and binding stoichiometry, as well as the determination of association constants. Advanced tools such as mass conservation analysis, multiwavelength analysis, and global analysis are introduced and discussed in the context of the experimental design. A detailed protocol guiding the investigator through the experimental steps and the data analysis is available as an internet resource. Curr. Protoc. Immunol. 79:18.8.1‐8.18.28. © 2007 by John Wiley & Sons, Inc.
Nanotechnological Aspects in Materials for Hydrogen Storage
Authors: Fichtner Maximilian
Published: November 2007

A nanotechnological approach aims at making tailor‐made developments for storage materials by combining different scientific disciplines and focussing on processes on the microscale. The approach has already been successful in achieving major advances in the field of novel solid materials for hydrogen storage. However, further breakthroughs are necessary to reach the goal of storage systems for fuel cell‐driven, mobile applications.
Separation Techniques for Carbon Nanotubes
Authors: Krupke Ralph , Hennrich Frank
Published: November 2007

Single‐walled carbon nanotubes have considerable potential as building blocks in future nanoscale electronics. The tubes exist in two modifications, metallic and semiconducting, which might be used in a range of electronic applications. One of the grand challenges is to separate metallic from semiconducting tubes in substantial quantities. In this respect, this article gives a brief overview over the separation techniques in this new field of nanotube research.
Analysis of the Hematopoietic Stem Cell Niche
Authors: Celso Cristina Lo , Klein Rachael J. , Scadden David T.
Published: November 2007

Hematopoietic stem cells (HSCs) continuously replenish all blood cell lineages not only to maintain the normal rapid turnover of differentiated cells but also to respond to injury and stress. Cell‐extrinsic mechanisms are critical determinants of the fine balance between HSC self‐renewal and differentiation. The bone marrow microenvironment has emerged as a new area of intense study to identify which of its many components constitute the HSC niche and regulate HSC fate. While HSCs have been isolated, characterized and used in clinical practice for many years thanks to the development of very specific assays and technology (i.e., bone marrow transplants and fluorescence activated cell sorting), study of the HSC niche has evolved by combining experimental designs developed in different fields. In this unit we describe a collection of protocols spanning a wide range of techniques that can help every researcher tackling questions regarding the nature of the HSC niche. Curr. Protoc. Stem Cell Biol. 3:2A.5.1‐2A.5.31. © 2007 by John Wiley & Sons, Inc.
Cloning of Large Positive‐Strand RNA Viruses
Authors: V. Peremyslov Valera , V. Dolja Valerian
Published: November 2007

Full‐length, biologically active cDNA clones of the positive‐strand RNA plant viruses are indispensable for investigating the functions of viral genes and control elements as well as generating virus‐derived gene expression and silencing vectors. Even though engineering of such clones for 4‐ to 10‐kb viral RNAs has become routine, it remains a challenging task for 15‐ to 20‐kb RNA genomes of the monopartite viruses in a family Closteroviridae. This unit describes strategic considerations and techniques used to generate an infectious cDNA clone of a closterovirus. The use of agroinfection to improve specific infectivity of the resulting clone is also explained. Curr. Protoc. Microbiol. 7:16F.1.1‐16F.1.26. © 2007 by John Wiley & Sons, Inc.
Fraser syndrome: A clinical study of 59 cases and evaluation of diagnostic criteria
Authors: van Haelst Mieke M. , Scambler Peter J. ,   Fraser Syndrome Collaboration Group, Hennekam Raoul C.M.
Published: November 2007

Fraser syndrome is an autosomal recessive congenital malformation syndrome characterized by cryptophthalmos, syndactyly, and urogenital defects. We studied the clinical features in 59 affected individuals from 40 families (25 consanguineous), and compared our findings to data from previous reviews. We found a higher frequency of abnormalities of the skull, larynx, umbilicus, urinary tract, and anus in our series of patients, and mental retardation and cleft lip with or without cleft palate were observed less frequently than previously reported. Clinical features in probands and sibs were remarkably similar. As can be expected prenatally diagnosed patients had more manifestations that gave rise to a pathological amount of amniotic fluid. Otherwise patients diagnosed before and after birth had similar frequencies of symptoms. Based on the present results we suggest an adaptation of diagnostic criteria for FS, including adding airway tract and urinary tract anomalies as major criteria. The specificity of the proposed diagnostic criteria was evaluated using the London Medical Database as a search tool. © 2007 Wiley‐Liss, Inc.
Acrofacial dysostosis syndrome type Rodriguez: Prenatal diagnosis and autopsy findings
Authors: Sermer David , Quercia Nada , Chong Karen , Chitayat David
Published: November 2007

A new lethal form of acrofacial dysostosis (AFD) syndrome was delineated by Rodriguez et al. [Rodriguez et al. (1990); Am J Med Genet 35:484–489]. We report on a male fetus with mandibulofacial dysostosis, including phocomelia‐like upper limb deficiencies and lower limb anomalies which are characteristic of AFD Rodriguez type. The diagnosis was made on prenatal sonogram at 20 and at 24.1 weeks gestation. The severity of the upper limb defects, the involvement of lower limbs, and the absence of eyelid coloboma and polythelia excluded the possibility of other conditions associated with acrofacial dysostosis (AFD) including Nager acrofacial dysostosis syndrome (NADS) and postaxial acrofacial dysostosis syndrome (POADS). This case further delineates the AFD syndrome type Rodriguez. © 2007 Wiley‐Liss, Inc.
Genetics of sexual development: A new paradigm
Authors: Blecher Stan R. , Erickson Robert P.
Published: November 2007

The classical paradigm of human and mammalian sexual development, largely based on work of Alfred Jost, depicts the genetic factor(s) that determine(s) sex as influencing only the fate of the gonad. A maleness factor produces testes (Primary Sex Determination). These organs secrete hormones which cause male Secondary Sexual Differentiation. In absence of the maleness factor, by default the gonad becomes an ovary, and the absence of testicular hormones leads to female secondary differentiation. In this article a new paradigm is proposed, to accommodate recent findings. Sexual dimorphism precedes gonadal development, in a Pregonadal Stage. Furthermore, female development is not by default—both male (Y) and female (X) sex‐chromosomal primary sex‐determining mechanisms probably exist. The human/mammalian male Y‐chromosomal sex‐determining gene is now known (SRY/Sry), and a candidate for a non‐inactivated, X‐linked, female determining factor, is under study. However, the proximate gonad‐determining genes are probably on autosomes. Pathways between the primary factors and the proximate gonad‐determining genes are indirect and complex. A hypothetical gene Z has been proposed, that inhibits the testis determiner and is itself the target of suppression by SRY/Sry. Candidates for proximate testis and ovary‐determining factors and for Z also exist. The “default” concept has also been superseded with respect to secondary sexual differentiation. Absence of testicular hormones does not produce a normal female phenotype; ovarian genes and hormones are necessary. Finally, sex‐chromosomal sex‐determining genes influence the development not only of non‐gonadal organs of secondary sexual development, but also of organs outside of the reproductive system. © 2007 Wiley‐Liss, Inc.
Neuroimaging findings in macrocephaly–capillary malformation: A longitudinal study of 17 patients
Authors: Conway Robert L. , Pressman Barry D. , Dobyns William B. , Danielpour Moise , Lee John , Sanchez‐Lara Pedro A. , Butler Merlin G. , Zackai Elaine , Campbell Lindsey , Saitta Sulagna C. , Clericuzio Carol L. , Milunsky Jeff M. , Hoyme H. Eugene , Shieh Joseph , Moeschler John B. , Crandall Barbara , Lauzon Julie L. , Viskochil David H. , Harding Brian , Graham John M.
Published: November 2007

Here, we report the neuroimaging findings and neurological changes in 17 unpublished patients with Macrocephaly–Capillary Malformation (M–CM). This syndrome has been traditionally known as Macrocephaly–Cutis Marmorata Telangiectatica Congenita (M–CMTC), but we explain why M–CM is a more accurate term for this overgrowth syndrome. We analyzed the 17 patients with available brain MRI or CT scans and compared their findings with features identified by a comprehensive review of published cases. White matter irregularities with increased signal on T2‐weighted images were commonly observed findings. A distinctive feature in more than half the patients was cerebellar tonsillar herniation associated with rapid brain growth and progressive crowding of the posterior fossa during infancy. In four such cases, we confirmed that the tonsillar herniation was an acquired event. Concurrently, with the development of these findings, ventriculomegaly (frequently obstructive) and dilated dural venous sinuses were observed in conjunction with prominent Virchow–Robin spaces in many of those in whom cerebellar tonsil herniation had developed. We postulate that this constellation of unusual features suggests a dynamic process of mechanical compromise in the posterior fossa, perhaps initiated by a rapidly growing cerebellum, which leads to congestion of the venous drainage with subsequently compromised cerebrospinal fluid reabsorption, all of which increases the posterior fossa pressure and leads to acquired tonsillar herniation. We make a distinction between congenital Chiari I malformation and acquired cerebellar tonsil herniation in this syndrome. We also observed numerous examples of abnormal cortical morphogenesis, including focal cortical dysplasia, polymicrogyria which primarily involved the perisylvian and insular regions, and cerebral and/or cerebellar asymmetric overgrowth. Other findings included a high frequency of cavum septum pellucidum or vergae, thickened corpus callosum, prominent optic nerve sheaths and a single case of venous sinus thrombosis. One patient was found to have a frontal perifalcine mass resembling a meningioma at age 5 years. This is the second apparent occurrence of this specific tumor in M–CM. © 2007 Wiley‐Liss, Inc.
Regional analysis on the occurrence of oral clefts in South America
Authors: Poletta F.A. , Castilla E.E. , Orioli I.M. , Lopez‐Camelo J.S.
Published: November 2007

The aim of this work was to search for unequal birth prevalence rates (BPRs) of cleft lip ± cleft palate (CL/P), and cleft palate only (CPO), among different geographic areas in South America, and to analyze phenotypic characteristics and associated risk factors in each identified cluster. Included were 5,128 CL/P cases, 1,745 CPO cases, and 3,712 controls (like‐sexed, non‐malformed liveborn infant, born immediately after a malformed one, in the same hospital), over 4,199,630 consecutive births. They were ascertained between 1967 and 2004, in 190 maternity hospitals of the ECLAMC (Estudio Colaborativo Latinoamericano de Malformaciones Congénitas) network, in 102 cities of all 10 South American countries. Non‐predefined geographical areas with significantly unusual cleft BPRs were identified with Kulldorf and Nagarwalla's spatial scan statistic, employing number of cases and births, and exact location of each hospital. Expected values were cleft BPRs registered for the entire ECLAMC hospital network. Syndromic and non‐syndromic clefts were considered for cluster analysis, and phenotypic characterization, while only non‐syndromic for risk factor analysis. Seven clusters for CL/P, and four for CPO, with unusual BPRs were identified. CL/P cases in high BPR areas were more severe than elsewhere in the sample, similar to a previous ECLAMC report on microtia. For CL/P, high BPR clusters were associated with high altitude above sea level, Amerindian ancestry, and low socioeconomic strata; low BPR clusters showed association with African Black ancestry. Advanced maternal age, a recognized risk factor for CPO, was also associated with the only identified geographic cluster for CPO. © 2007 Wiley‐Liss, Inc.
Muenke syndrome (FGFR3‐related craniosynostosis): Expansion of the phenotype and review of the literature
Authors: Doherty Emily S. , Lacbawan Felicitas , Hadley Donald W. , Brewer Carmen , Zalewski Christopher , Kim H. Jeff , Solomon Beth , Rosenbaum Kenneth , Domingo Demetrio L. , Hart Thomas C. , Brooks Brian P. , Immken LaDonna , Lowry R. Brian , Kimonis Virginia , Shanske Alan L. , Jehee Fernanda Sarquis , Bueno Maria Rita Passos , Knightly Carol , McDonald‐McGinn Donna , Zackai Elaine H. , Muenke Maximilian
Published: November 2007

Muenke syndrome is an autosomal dominant disorder characterized by coronal suture craniosynostosis, hearing loss, developmental delay, carpal and tarsal fusions, and the presence of the Pro250Arg mutation in the FGFR3 gene. Reduced penetrance and variable expressivity contribute to the wide spectrum of clinical findings in Muenke syndrome. To better define the clinical features of this syndrome, we initiated a study of the natural history of Muenke syndrome. To date, we have conducted a standardized evaluation of nine patients with a confirmed Pro250Arg mutation in FGFR3. We reviewed audiograms from an additional 13 patients with Muenke syndrome. A majority of the patients (95%) demonstrated a mild‐to‐moderate, low frequency sensorineural hearing loss. This pattern of hearing loss was not previously recognized as characteristic of Muenke syndrome. We also report on feeding and swallowing difficulties in children with Muenke syndrome. Combining 312 reported cases of Muenke syndrome with data from the nine NIH patients, we found that females with the Pro250Arg mutation were significantly more likely to be reported with craniosynostosis than males (P < 0.01). Based on our findings, we propose that the clinical management should include audiometric and developmental assessment in addition to standard clinical care and appropriate genetic counseling. Published 2007 Wiley‐Liss, Inc.
A study of 534 fetal pathology cases from prenatal diagnosis referrals analyzed from 1989 through 2000
Authors: Laury Anna , Sanchez‐Lara Pedro A. , Pepkowitz Samuel , Graham John M.
Published: November 2007

Useful and meaningful counseling following the discovery of a fetal loss or fetal anomalies requires an accurate diagnosis. In order to achieve this, our center utilized a multidisciplinary approach which included an ultrasonographer, perinatologist, genetic counselor, dysmorphologist, clinical geneticist, and pathologist. In this article, we report our experience with the analysis and evaluation of 534 cases seen between 1989 and 2000. In total, we were able to identify the cause of fetal loss in 369 cases (69%). In 98/369 cases (18.4%) the condition was attributed to a Mendelian process and in 78 (14.6%), to chromosomal abnormalities. The overall average maternal age was 31.6 years, the maternal age in cases of chromosomal abnormality was slightly higher (33.3 years). Our findings described below reiterate the clinical usefulness of a team specifically trained in the approach to fetal loss and/or fetal anomalies. © 2007 Wiley‐Liss, Inc.
Nonsense‐mediated decay and the molecular pathogenesis of mutations in SALL1 and GLI3
Authors: Furniss Dominic , Critchley Paul , Giele Henk , Wilkie Andrew O.M.
Published: November 2007

Mutations in SALL1 and GLI3 are responsible for human limb malformation syndromes. The molecular pathophysiology of these mutations is incompletely understood, and many conclusions have been drawn from studies performed in the mouse. We identified truncating mutations in SALL1 and GLI3 in patients with limb malformation and studied the contribution of nonsense‐mediated decay (NMD) to the expression of mutant mRNA in patient‐derived fibroblasts. Quantification of the relative proportions of mutant and wild‐type alleles was performed by pyrosequencing. In SALL1, a mutant allele causing Townes–Brocks syndrome was unexpectedly resistant to NMD, whereas a different mutation causing a much milder phenotype was susceptible to NMD. In GLI3, all three mutant alleles tested were susceptible to NMD. This work provides novel insights into the molecular pathophysiology of SALL1 and GLI3 mutations, extends the phenotypic spectrum of SALL1 mutations, and provides an example of a human mutation which does not follow the usual accepted positional rules governing mammalian NMD. © 2007 Wiley‐Liss, Inc.