Enhanced stability of layered phases in parallel hard-spherocylinders due to the addition of hard spheres

There is increasing evidence that entropy can induce microphase separation in binary fluid mixtures interacting through hard particle potentials. One such phase consists of alternating two dimensional liquid-like layers of rods and spheres. We study the transition from a uniform miscible state to this ordered state using computer simulations and compare results to experiments and theory. We conclude that (1) there is stable entropy driven microphase separation in mixtures of parallel rods and spheres, (2) adding spheres smaller then the rod length decreases the total volume fraction needed for the formation of a layered phase, therefore small spheres effectively stabilize the layered phase; the opposite is true for large spheres and (3) the degree of this stabilization increases with increasing rod length.Comment: 11 pages, 9 figures. Submitted to Phys. Rev. E. See related website http://www.elsie.brandeis.ed

Differential diagnosis in spinal and bulbar muscular atrophy clinical and molecular aspects

Kennedy disease is caused by an enlarged trinucleotide repeat sequence within the androgen receptor gene. We report here seven male patients with a benign motor neuron syndrome highly analogous to Kennedy disease but with a normal trinucleotide repeat

Polydisperse star polymer solutions

We analyze the effect of polydispersity in the arm number on the effective interactions, structural correlations and the phase behavior of star polymers in a good solvent. The effective interaction potential between two star polymers with different arm numbers is derived using scaling theory. The resulting expression is tested against monomer-resolved molecular dynamics simulations. We find that the theoretical pair potential is in agreement with the simulation data in a much wider polydispersity range than other proposed potentials. We then use this pair potential as an input in a many-body theory to investigate polydispersity effects on the structural correlations and the phase diagram of dense star polymer solutions. In particular we find that a polydispersity of 10%, which is typical in experimental samples, does not significantly alter previous findings for the phase diagram of monodisperse solutions.Comment: 14 pages, 7 figure

The clinical spectrum of limb girdle muscular dystrophy. A survey in the Netherlands

A cross-sectional study was performed in the Netherlands to define the clinical characteristics of the various subtypes within the broad and heterogeneous entity of limb girdle muscular dystrophy (LGMD). An attempt was made to include all known cases of LGMD in the Netherlands. Out of the reported 200 patients, 105 who fulfilled strictly defined criteria were included. Forty-nine patients, mostly suffering from dystrophinopathies and facioscapulohumeral muscular dystrophy, appeared to be misdiagnosed. Thirty-four cases were sporadic, 42 patients came from autosomal recessive and 29 from autosomal dominant families. The estimated prevalence of LGMD in the Netherlands was at least 8.1 x 10-6. The clinical features of the autosomal recessive and sporadic cases were indistinguishable from those of the autosomal dominant patients, although half hypertrophy was seen more frequently, and the course of the disease was more severe in autosomal recessive and sporadic cases. The pectoralis, iliopsoas and gluteal muscles, hip adductors and hamstrings were the most affected muscles. Distal muscle involvement occurred late in the course of the disease. Facial weakness was a rare phenomenon. The severity of the clinical picture was correlated with a deteriorating lung function. All autosomal dominantly inherited cases showed a mild course, although in two families life-expectancy was reduced because of concomitant cardiac involvement

Hypokinesia and presenile dementia in a Dutch family with a novel insertion in the prion protein gene

Contains fulltext : 22242.PDF (publisher's version ) (Open Access

Action of lovastatin, simvastatin, and pravastatin on sterol synthesis and their antiproliferative effect in cultured myoblasts from human striated muscle

Lovastatin, simvastatin, and pravastatin are fairly strong inhibitors of sterol synthesis in human myoblasts in culture. Lovastatin and simvastatin have IC50 values of 19 ± 6 nM and 4.0 ± 2.3 nM, respectively. Pravastatin is a weaker inhibitor of sterol synthesis (IC50 value of 110 ± 38 nM). Through inhibition of mevalonate production, these compounds have a distinct inhibiting effect on cell proliferation. Because proliferation of myoblasts is important in the repair of damaged skeletal muscle, experiments were performed to investigate the effect of lovastatin, simvastatin, and pravastatin on cell proliferation and cell viability. The more potent inhibitors of sterol synthesis, lovastatin, and simvastatin, were able to inhibit the proliferation of these cells during 3 days of incubation with drug concentrations of 1 μM for lovastatin and 0.1 μM or 1 μM for simvastatin. DNA synthesis was decreased by more than 80% in the presence of 1 μM of lovastatin or simvastatin. In contrast, under these conditions, pravastatin had no influence on cell proliferation or DNA synthesis, which is probably related to the lack of inhibition of sterol synthesis by pravastatin on extended incubation. The three 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors did not disturb cell viability because mitochondrial dehydrogenase activity and ATP content remained proportional to the number of cells in the culture at any concentration used. Chemicals/CAS: Acetic Acid, 64-19-7; Adenosine Triphosphate, 56-65-5; Anticholesteremic Agents; DNA, 9007-49-2; Enzyme Inhibitors; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Lovastatin, 75330-75-5; Oxidoreductases, EC 1.-; Pravastatin, 81093-37-0; Simvastatin, 79902-63-9; Sterol

Utenriksdepartementet og frivillige organisasjoners nødhjelpssamarbeid : En analyse av frivillige organisasjoners autonomi

Korporatismeforskningen har hovedsakelig vært opptatt av beslutningsmakt. Denne studien av Utenriksdepartementet og frivillige organisasjoners samarbeid om nødhjelp viser at samspillet ikke kan sees så mye i lys av interessehevding og forlikelse mellom motstridende interesser, som i lys av idé- og definisjonsmakt. Korporatisme har tradisjonelt vært fortolket ved hjelp av de to konkurrerende forestillingene om interessehevding versus styringskapasitet. På grunn av høy grad av felles interesser har interessehevding begrenset forklaringskraft på bistandsfeltet. Studien benytter to nødhjelpsaksjoner for å analysere tilpassningsgrad og autonomi i organisasjonene, og om den ene nødhjelpsaksjonen var mer politisk styrt enn den andre. Min undersøkelse har vist at UD er en stor maktfaktor innen norsk humanitærhjelp, og en ubestridt forvalter av Norges nødhjelpsinnsats. Organisasjonenes tilpassning til statlig politikk er betydelig. Jeg har analysert maktrelasjonene mellom UD og organisasjonene ved hjelp av tre maktperspektiver. Jeg har funnet at, i tillegg til at den tradisjonelle forståelsen av makt er nyttig, er Steven Lukes’ tredje maktperspektiv vel så fruktbart. Først og fremst fordi mangel på konflikt ikke trenger bety at makt ikke utøves eller at organisasjonenes beslutninger er autonome. Studien viser at Røde Kors og Kirkens Nødhjelp hadde noe begrenset autonomi i de to casene, og at autonomien begrenses ytterligere ved caser hvor de politiske prioriteringene blir viktigere enn de humanitære. På den annen side kan organisasjonene øve innflytelse på bistandspolitikken. Påvirkningen går begge veier. Studien viser at organisasjonene i liten grad føler at deres prioriteringer er begrenset av statlige føringer, og at dette kan skyldes at departementets autoritetssituasjon har en form som kan tolkes i ved hjelp av det tredje maktperspektivet. Mitt argument er at organisasjonenes autonomi varierer fra case til case. Den er reell så lenge det er felles interesser og så lenge det passer staten. Det har vokst fram en kontraktskultur mellom aktørene, og oppgaven viser at dette kan tolkes som statlig ansvarsfraskrivelse. Studien viser også at nødhjelpsbehovene i Irak i 2003 var overfokuserte og at organisasjonene brukte sine resurser galt