Neurological manifestations and molecular genetics of acute intermittent porphyria in North Western Russia

Acute intermittent porphyria (AIP, MIM #176000) is an inherited metabolic disease due to a partial deficiency of the third enzyme, hydroxymethylbilane synthase (HMBS, EC: 4.3.1.8), in the haem biosynthesis. Neurological symptoms during an acute attack, which is the major manifestation of AIP, are variable and relatively rare, but may endanger a patient's life. In the present study, 12 Russian and two Finnish AIP patients with severe neurological manifestations during an acute attack were studied prospectively from 1995 to 2006. Autonomic neuropathy manifested as abdominal pain (88%), tachycardia (94%), hypertension (75%) and constipation (88%). The most common neurological sign was acute motor peripheral neuropathy (PNP, 81%) often associated with neuropathic sensory loss (54%) and CNS involvement (85%). Despite heterogeneity of the neurological manifestations in our patients with acute porphyria, the major pattern of PNP associated with abdominal pain, dysautonomia, CNS involvement and mild hepatopathy could be demonstrated. If more strict inclusion criteria for biochemical abnormalities (>10-fold increase in excretion of urinary PBG) are applied, neurological manifestations in an acute attack are probably more homogeneous than described previously, which suggests that some of the neurological patients described previously may not have acute porphyria but rather secondary porphyrinuria. Screening for acute porphyria using urinary PBG is useful in a selected group of neurological patients with acute PNP or encephalopathy and seizures associated with pain and dysautonomia. Clinical manifestations and the outcome of acute attacks were used as a basis for developing a 30-score scale of the severity of an acute attack. This scale can easily be used in clinical practice and to standardise the outcome of an attack. Degree of muscle weakness scored by MRC, prolonged mechanical ventilation, bulbar paralysis, impairment of consciousness and hyponatraemia were important signs of a poor prognosis. Arrhythmia was less important and autonomic dysfunction, severity of pain and mental symptoms did not affect the outcome. The delay in the diagnosis and repeated administrations of precipitating factors were the main cause of proceeding of an acute attack into pareses and severe CNS involvement and a fatal outcome in two patients. Nerve conduction studies and needle EMG were performed in eleven AIP patients during an acute attack and/or in remission. Nine patients had severe PNP and two patients had an acute encephalopathy but no clinically evident PNP. In addition to axonopathy, features suggestive of demyelination could be demonstrated in patients with severe PNP during an acute attack. PNP with a moderate muscle weakness was mainly pure axonal. Sensory involvement was common in acute PNP and could be subclinical. Decreased conduction velocities with normal amplitudes of evoked potentials during acute attacks with no clinically evident PNP indicated subclinical polyneuropathy. Reversible symmetrical lesions comparable with posterior reversible encephalopathy syndrome (PRES) were revealed in two patients' brain CT or MRI during an acute attack. In other five patients brain MRI during or soon after the symptoms was normal. The frequency of reversible brain oedema in AIP is probably under-estimated since it may be short-lasting and often indistinguishable on CT or MRI. In the present study, nine different mutations were identified in the HMBS gene in 11 unrelated Russian AIP patients from North Western Russia and their 32 relatives. AIP was diagnosed in nine symptom-free relatives. The majority of the mutations were family-specific and confirmed allelic heterogeneity also among Russian AIP patients. Three mutations, c.825+5G>C, c.825+3_825+6del and c.770T>C, were novel. Six mutations, c.77G>A (p.R26H), c.517C>T (p.R173W), c.583C>T (p.R195C), c.673C>T (p.R225X), c.739T>C (p.C247R) and c.748G>C (p.E250A), have previously been identified in AIP patients from Western and other Eastern European populations. The effects of novel mutations were studied by amplification and sequencing of the reverse-transcribed total RNA obtained from the patients' lymphoblastoid or fibroblast cell lines. The mutations c.825+5G>C and c.770T>C resulted in varyable amounts of abnormal transcripts, r.822_825del (p.C275fsX2) and [r.770u>c, r.652_771del, r.613_771del (p.L257P, p.G218_L257del, p.I205_L257del)]. All mutations demonstrated low residual activities (0.1-1.3 %) when expressed in COS-1 cells confirming the causality of the mutations and the enzymatic defect of the disease. The clinical outcome, prognosis and correlation between the HMBS genotype and phenotype were studied in 143 Finnish and Russian AIP patients with ten mutations (c.33G>T, c.97delA, InsAlu333, p.R149X, p.R167W, p.R173W, p.R173Q, p.R225G, p.R225X, c.1073delA) and more than six patients in each group. The patients were selected from the pool of 287 Finnish AIP patients presented in a Finnish Porphyria Register (1966-2003) and 23 Russian AIP patients (diagnosed 1995-2003). Patients with the p.R167W and p.R225G mutations showed lower penetrance (19% and 11%) and the recurrence rate (33% and 0%) in comparison to the patients with other mutations (range 36 to 67% and 0 to 66%, respectively), as well as milder biochemical abnormalities [urinary porphobilinogen 47±10 vs. 163±21 mol/L, p<0.001; uroporphyrin 130±40 vs. 942±183 nmol/L, p<0.001] suggesting a milder form of AIP in these patients. Erythrocyte HMBS activity did not correlate with the porphobilinogen excretion in remission or the clinical of the disease. In all AIP severity patients, normal PBG excretion predicted freedom from acute attacks. Urinary PBG excretion together with gender, age at the time of diagnosis and mutation type could predict the likelihood of acute attacks in AIP patients.

Effects of pot fishing on the physical condition of snow crab (Chionoecetes opilio) and southern Tanner crab (Chionoecetes bairdi)

The effects of commercial fishing with crab pots on the physical condition of the snow crab (Chionoecetes opilio) and southern Tanner crab (C. bairdi) were investigated in the Bering Sea and in Russian waters of the Sea of Okhotsk. In crabs that were subjected to pot hauling, the presence of gas embolism and the deformation of gill lamellae were found in histopathological investigations. Crab vitality, which was characterized subjectively through observation of behavioral responses, depended on not only the number of pot hauls but also the time between hauls. Immediately after repeated pot hauls at short time intervals (≤3 days), we observed a rapid decline in vitality of crabs. When hauling intervals were increased to >3 days, the condition of crabs did not significantly change. After repeated pot hauls, concentration of the respiratory pigment hemocyanin ([Hc]) was often lower in the hemolymph of crabs than in the hemolymph of freshly caught animals. Our research indicated that changes in [Hc] in crabs after repeated pot hauls were caused by the effects of decompression and not by starvation of crabs in pots or exposure of crabs to air. We suggest that the decrease in [Hc] in hemolymph of snow and southern Tanner crabs was a response to the adverse effects of decompression and air-bubble disease. The decrease in [Hc] in affected crabs may be a result of mechanisms that regulate internal pressure in damaged gills to optimize respiratory circulation.

The stripe-backed weasel Mustela strigidorsa: taxonomy, ecology, distribution and status

1. The stripe-backed weasel Mustela strigidorsa is one of the rarest and least-known mustelids in the world. Its phylogenetic relationships with other Mustela species remain controversial, though several unique morphological features distinguish it from congeners. 2. It probably lives mainly in evergreen forests in hills and mountains, but has also been recorded from plains forest, dense scrub, secondary forest, grassland and farmland. Known sites range in altitude from 90 m to 2500 m. Data are insufficient to distinguish between habitat and altitudes which support populations, and those where only dispersing animals may occur. 3. It has been confirmed from many localities in north-east India, north and central Myanmar, south China, north Thailand, north and central Laos, and north and central Vietnam. Given the limited survey effort, the number of recent records shows that the species is not as rare as hitherto believed. Neither specific nor urgent conservation needs are apparent.

“Hegemony Protected by the Armour of Coercion”: Dashiell Hammett’s Red Harvest and the State

This article argues that Dashiell Hammett's 1929 novel Red Harvest is best understood in the context of the consolidation and expansion of the US state following the First World War and the Russian Revolution. It also argues that Hammett's novel constitutes a highly significant articulation of theoretical debates about the nature of political authority and state power in the modern era and speaks about the transition of one state formation to another. Insofar as Red Harvest explores the way in which the state's coercive and ethical character are bound up together, this article argues that Hammett's novel draws upon an understanding of political authority and state power primarily derived from Gramsci, via Marx. Gramsci insists that control cannot be maintained through force alone (and his conception of hegemony, in turn, suggests a power bloc that can become fragmented and disunited in a war of position). In the same way, Red Harvest traces the transformation of the “economic-corporate” state into the expanded or “ethical” State but crucially any ethical dimension, as Gramsci notes, is always beholden to the needs of the capitalist economy. As such, the apparently arbitrary bloodshed in the novel is conceived as a relatively minor realignment in the ranks of the capitalist classes – certainly less serious than the miners' strike that prefigures the novel. What makes this realignment significant is that it calls attention to the state both as repressive and as a site of conflict and compromise. Here, the work performed by the Continental Op and by the crime novel in general – simultaneously buttressing and, to some extent, contesting the power of the state – needs to be understood as part of the process by which the state is consistently enacting hegemony (albeit protected by the armour of coercion). The article concludes by pointing out that while Gramsci is perhaps too willing to dwell upon the state's expanded reach, Red Harvest is more interested in examining possible “cracks and fissures” in the state formation, even if the critique it ultimately offers goes nowhere and yields nothing.

A Contested Landscape: Monuments, public memory, and post-Soviet Identity in Stavropol’, Russia

Much recent literature in cultural, political and social geography has considered the relationship between identity, memory, and the urban landscape. This paper interrogates such literature through exploring the complex materialisation of memorialisation in post-Soviet Russia. Using the example of the statue of General Alexei Ermolov in Stavropol', an analysis of the cityscape reveals interethnic tensions over differing interpretations of the life and history of the person upon whom the statue is based. The existence of a rich literature on Ermolov and the Russian colonial experience in the North Caucasus helps to explain this. The symbolic cityscape of Stavropol' plays an important role in interethnic relations in the multi-ethnic city; it is both an arena through which Russian identity is communicated with people and produced and reproduced, and an arena through which Russian citizens compete with each other for authority on historical narratives that operate at and between a number of scales. People's readings of the cityscape can reveal much about power and space in contemporary Russia.

On Extraterritoriality and the Gazprom case

In September 2012 the European Commission opened a formal investigation of Gazprom’s business practices in the EU. The Gazprom investigation raises a number of questions. One of them is the issue of jurisdiction—whether EU competition law applies to Gazprom, a foreign company. A day after the opening of the proceeding, in a press note and alongside other issues, Gazprom raised the question of jurisdiction, noting that it complies with laws of the countries in which it operates and that is it ‘registered outside the jurisdiction of the EU’. This statement, possibly, prompted some commentators to consider the applicability of EU law in this case.
In a piece in the ECLR, entitled ‘Iron Curtain at the border: Gazprom and the Russian blocking order to prevent the extraterritoriality of EU competition law’, Sean Morris offered his views on some of the aspects of the Gazprom case, including the issue of jurisdiction. Morris discussed also the blocking Order issued by the Russian President in response to the European Commission’s investigation, and its possible effects in the Gazprom case.
This article seeks to add a few important and relevant issues of law relating to extraterritoriality and the reach of EU law generally and in particular— in the context of the Gazprom investigation and in the light of the Morris article. This piece also sheds some light on the considerations which might have informed Russia’s hastily enactment of the Blocking Order.

Gas-phase kinetic and theoretical studies of reactions of germylenes and dimethylstannylene

The results of time-resolved gas phase studies of labile germylenes (GeH2 and GeMe2) and dimethylstannylene (SnMe2) reactions reported to date are considered together with data of quantum-chemical investigations of the potential energy surfaces of these systems. Reaction mechanisms are discussed. A comparison of reactivity in the series of carbene analogs, ER2 (E = Si, Ge, Sn, R = H, Me), is made.

First observation of the Cabibbo-suppressed decays Xi(+)(c)->Sigma(+)pi(-)pi(+) and Xi(+)(c)->Sigma(-)pi(+)pi(+) and measurement of their branching ratios

We report the first observation of two Cabibbo-suppressed ecay modes, Xi(+)(c) -> Sigma(+)pi(-)pi(+) and Xi c+ -> Sigma(-)pi(+)pi(+). We observe 59 +/- 14 over a background of 87, and 22 +/- 8 over a background of 13 events, respectively, for the signals. The data were accumulated using the SELEX spectrometer during the 1996-1997 fixed target run at Fermilab, chiefly from a 600 Gev/c Sigma(-) beam. The branching ratios of the decays relative to the Cabibbo-favored Xi c+ -> Xi(-)pi(+)pi(+) are measured to be B(Xi(+)(c) -> Sigma(+)pi(-)pi(+))/B(Xi(+)(c) -> Xi(-)pi(+)pi(+)) = 0.48 +/- 0.20, and B(Xi(+)(c) -> Sigma(-)pi(+)pi(+))/B(Xi(+)(c) -> Sigma(-)pi(+)pi(+)) = 0.18 +/- 0.09, respectively. We also report branching ratios for the same decay modes of the Delta(+)(c) relative to Delta(+)(c) -> pK(-)pi(+.) (C) 2008 Elsevier B.V. All rights reserved.

CONSTRUCTING QUANTUM OBSERVABLES AND SELF-ADJOINT EXTENSIONS OF SYMMETRIC OPERATORS. III. SELF-ADJOINT BOUNDARY CONDITIONS

This paper completes the review of the theory of self-adjoint extensions of symmetric operators for physicists as a basis for constructing quantum-mechanical observables. It contains a comparative presentation of the well-known methods and a newly proposed method for constructing ordinary self-adjoint differential operators associated with self-adjoint differential expressions in terms of self-adjoint boundary conditions. The new method has the advantage that it does not require explicitly evaluating deficient subspaces and deficiency indices (these latter are determined in passing) and that boundary conditions are of explicit character irrespective of the singularity of a differential expression. General assertions and constructions are illustrated by examples of well-known quantum-mechanical operators like momentum and Hamiltonian.

Center of mass energy and system-size dependence of photon production at forward rapidity at RHIC

We present the multiplicity and pseudorapidity distributions of photons produced in Au + Au and Cu + Cu collisions at root(s)NN = 62.4 and 200 GeV. The photons are measured in the region -3.7 < eta < -2.3 using the photon Multiplicity detector in the STAR experiment at RHIC. The number of photons produced per average number of participating nucleon pairs increases with the beam energy and is independent of (lie collision centrality. For collisions with similar average numbers of participating nucleons the photon multiplicities are observed to be similar for An + Au and Cu + Cu collisions at a given beam energy. The ratios of the number of charged particles to photons in the measured pseudorapidity range are found to be 1.4 +/- 0.1 and 1.2 +/- 0.1 for root(s)NN = 62.4 and 200 GeV, respectively. The energy dependence of this ratio could reflect varying contributions from baryons to charged particles, while mesons are the dominant contributors to photon production in the given kinematic region. The photon pseudorapidity distributions normalized by average number of participating nucleon pairs, when plotted as a function of eta-Y(beam), are found to follow a longitudinal scaling independent of centrality and colliding ion species at both beam energies. (C) 2009 Elsevier B.V. All rights reserved.

Energy and system size dependence of phi meson production in Cu plus Cu and Au plus Au collisions

We study the beam-energy and system-size dependence of phi meson production (using the hadronic decay mode phi -> K(+) K(-)) by comparing the new results from Cu + Cu collisions and previously reported Au + Au collisions at root s(NN) = 62.4 and 200 GeV measured in the STAR experiment at RHIC. Data presented in this Letter are from mid-rapidity (vertical bar y vertical bar < 0.5) for 0.4 < p(T) < 5 GeV/c. At a given beam energy, the transverse momentum distributions for phi mesons are observed to be similar in yield and shape for Cu + Cu and Au + Au colliding systems with similar average numbers of participating nucleons. The phi meson yields in nucleus-nucleus collisions, normalized by the average number of participating nucleons, are found to be enhanced relative to those from p + p collisions. The enhancement for phi mesons lies between strange hadrons having net strangeness = 1 (K(-) and <(A)over bar>) and net strangeness = 2 (Xi). The enhancement for phi mesons is observed to be higher at root s(NN) = 200 GeV compared to 62.4 GeV. These observations for the produced phi(s (s) over bar) mesons clearly suggest that, at these collision energies, the source of enhancement of strange hadrons is related to the formation of a dense partonic medium in high energy nucleus-nucleus collisions and cannot be alone due to canonical suppression of their production in smaller systems. (C) 2009 Elsevier B.V. All rights reserved.

Understanding Foreign Masters : the examples of Rabindranath Tagore and Fyodor Dostoyevskiy

The Indian author Rabindranath Tagore was received like royalty during his visits to the West after winning the Nobel Prize in 1913. Dreams of foreign cultures offered a retreat from a complicated age. In a time when the West appeared to be living under threat of disintegration and when industrialism seemed like a cul-de-sac, he appeared to offer the promise of a return to a lost paradise, a spiritual abode that is superior to the restless Western culture. However, Tagore’s popularity faded rapidly, most notably in England, the main target of his criticism. Soon after Tagore had won the Nobel Prize, the English became indignant at Tagore’s anti-colonial attitude.Tagore visited Sweden in 1921 and 1926 and was given a warm reception. His visits to Sweden can be seen as an episode in a longer chain of events. It brought to life old conceptions of India as the abode of spirituality on earth. Nevertheless, interest in him was a relatively short-lived phenomenon in Sweden. Only a few of his admirers in Sweden appreciated the complexity of Tagore’s achievements. His “anathema of mammonism”, as a Swedish newspaper called it, was not properly received. After a steady stream of translations his popularity flagged towards the end of the 1920s and then almost disappeared entirely. Tagores visits in Sweden gave an indication that India was on the way to liberate itself from its colonial legacy, which consequently contributed to the waning of his popularity in the West. In the long run, his criticism of the drawbacks in the western world became too obvious to maintain permanent interest. The Russian author Fyodor Dostoyevskiy’s Crime and Punishment (1866) has enticed numerous interpretations such as the purely biographical approach. In the nervous main character of the novel, the young student Raskolnikov, one easily recognizes Dostoyevskiy himself. The novel can also be seen as a masterpiece of realistic fiction. It gives a broad picture of Saint Petersburg, a metropolis in decay. Crime and Punishment can also be seen as one of the first examples of a modern psychological novel, since it is focused on the inner drama of its main character, the young student Raskolnikov. His actions seem to be governed by mere coincidences, dreams and the spur of the moment. it seems fruitful to study the novel from a psychoanalytical approach. In his book Raskolnikov: the way of the divided towards unity in Crime and Punishment (1982), a Swedish scholar, Owe Wikström, has followed this line of interpretation all the way to Freud’s disciple C G Jung. In addition to this, the novel functions as an exciting crime story. To a large extent it is Viktor Sjklovskij and other Russian formalists from the 1920s and onwards who have taught the western audience to understand the specific nature of the crime story. The novel could be seen as a story about religious conversion. Like Lasarus in the Bible (whose story attracts a lot of attention in the novel) Raskolnikov is awakened from the dead, and together with Sonja he starts a completely new life. The theme of conversion has a special meaning for Dostoyevskiy. For him the conversion meant an acknowledgement of the specific nature of Russia itself. Crime and punishment mirrors the conflict between traditional Russian values and western influences that has been obvious in Russia throughout the history of the country. The novel reflects a dialogue that still continues in Russian society. The Russian literary historian Mikhail Bakhtin, who is probably the most famous interpreter of the works of Dostoyevskiy, has become famous precisely by emphasizing the importance of dialogues in novels like Crime and Punishment. According to Bakhtin, this novel is characterized by its multitude of voices. Various ideas are confronted with each other, and each one of them is personified by one of the characters in the novel. The author has resigned from his position as the superior monitor of the text, and he leaves it to the reader to decide what interpretation is the correct one..The aim of the present study is thus to analyze the complex reactions in the west to Tagore’s visits in Sweden and to Fyodor Dostoyevskiys novel Crime and Punishment.. This leads to more general conclusions on communication between cultures.

Underlying Event measurements in pp collisions at root s=0.9 and 7 TeV with the ALICE experiment at the LHC

We present measurements of Underlying Event observables in pp collisions at root s = 0 : 9 and 7 TeV. The analysis is performed as a function of the highest charged-particle transverse momentum p(T),L-T in the event. Different regions are defined with respect to the azimuthal direction of the leading (highest transverse momentum) track: Toward, Transverse and Away. The Toward and Away regions collect the fragmentation products of the hardest partonic interaction. The Transverse region is expected to be most sensitive to the Underlying Event activity. The study is performed with charged particles above three different p(T) thresholds: 0.15, 0.5 and 1.0 GeV/c. In the Transverse region we observe an increase in the multiplicity of a factor 2-3 between the lower and higher collision energies, depending on the track p(T) threshold considered. Data are compared to PYTHIA 6.4, PYTHIA 8.1 and PHOJET. On average, all models considered underestimate the multiplicity and summed p(T) in the Transverse region by about 10-30%.

Longitudinal and transverse spin asymmetries for inclusive jet production at mid-rapidity in polarized p+p collisions at root s=200 GeV

We report STAR measurements of the longitudinal double-spin asymmetry A(LL), the transverse singlespin asymmetry A(N), and the transverse double-spin asymmetries A(Sigma) and A(TT) for inclusive jet production at mid-rapidity in polarized p + p collisions at a center-of-mass energy of root s = 200 GeV. The data represent integrated luminosities of 7.6 pb(-1) with longitudinal polarization and 1.8 pb(-1) with transverse polarization, with 50%-55% beam polarization, and were recorded in 2005 and 2006. No evidence is found for the existence of statistically significant jet A(N), A(Sigma), or A(TT) at mid-rapidity. Recent model calculations indicate the A(N) results may provide new limits on the gluon Sivers distribution in the proton. The asymmetry A(LL) significantly improves the knowledge of gluon polarization in the nucleon.