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Laminins and Congenital Muscular Dystrophy - From a Mouse Model to Gene Therapy of Laminin alpha2 chain deficiency?

Gene therapy holds great promise for treating many genetic diseases, including muscular dystrophies. Mutations in the gene encoding laminin alpha2 chain ? an extracellular protein prominently expressed in the neuromuscular system ? cause a severe neuromuscular disorder: congenital muscular dystrophy type 1A (MDC1A). Currently, there is no treatment for MDC1A. Preclinical studies are the first step

Utilization of primary care versus specialized care in children with and without chronic illness. A population-based study

Children's utilization of curative care was studied to analyse the division of responsibilities between undifferentiated primary care and specialized care. All chronically ill (n = 510), a control group (n = 287) and the total population 0-15 years of age (n = 6080) in a primary care district were studied using register data. Chronically ill children comprised 8.4% of the total child population an

Multiplexed predictive coding of speech

In this paper we present a novel method for predictive coding with application to transmission of speech over packet-switched networks. Our method uses multiplexing to distribute a part of the information about a segment of each speech signal in several data packets while keeping the data packet rate and payload for that part of the information unchanged. We investigate three multiplexing schemes:

Design of mixed MPLS/OSPF routing in IP networks

In this paper, we address some Internet core network optimization issues related to a combination of Open Shortest Path First (OSPF) and Multi Protocol Label Switching (MPLS) routing protocols. Since MPLS can be combined with any routing protocol there has appeared an idea of coupling IP streams routed via the OSPF protocol and MPLS Label Switched Paths (LSPs) in the same network. One expected adv

Search for resonant (nu)over-tilde production at root s=183 to 208 GeV

Searches for resonant (ν) over tilde production in e(+)e(-) collisions under the assumption that R-parity is not conserved and that the dominant R-parity violating coupling is lambda(121) or lambda(131) used data recorded by DELPHI in 1997 to 2000 at centre-of-mass energies of 183 to 208 GeV. No deviation from the Standard Model was observed. Upper limits are given for the lambda(121) and lambda(1

Cdk6 blocks myeloid differentiation by interfering with Runx1 DNA binding and Runx1-C/EBP alpha interaction

Interactions between the cell cycle machinery and transcription factors play a central role in coordinating terminal differentiation and proliferation arrest. We here show that cyclin-dependent kinase 6 (Cdk6) is specifically expressed in proliferating hematopoietic progenitor cells, and that Cdk6 inhibits transcriptional activation by Runx1, but not C/ EBP alpha or PU. 1. Cdk6 inhibits Runx1 acti

Charge sampling analogue FIR filter

The finite impulse response (FIR) function of the proposed filter is realised by integrating weighted signal currents in a given time window on a capacitor. The resulting capacitor voltage becomes the filter output, periodically available between integration and reset. In such an FIR filter, the hardware cost is delinked to the number of taps,

Bioelectrocatalytical detection of H2O2 with different forms of horseradish peroxidase directly adsorbed at polycrystalline silver and gold

The bioelectrocatalytical reduction of H2O2 based on direct electron transfer (ET) between polycrystalline silver and the heme containing active site of horseradish peroxidase (HRP) is studied and compared with that obtained at gold. Native HRP and recombinant wild type HRP, containing additionally a six-histidine tag at the N-terminus, NHisrHRP, have been used for adsorptive modification of the e

No evidence of genetic differentiation between lesser redpolls Carduelis flammea cabaret and common redpolls Carduelis f. flammea.

The remarkable variation in plumage and morphological characters in the redpoll complex Carduelis flammea-hornemanni has puzzled taxonomists for several decades. In contrast, molecular studies have not revealed any genetic differentiation among the phenotypic redpoll forms. This could either be a result of high present-day gene flow or morphological differentiation following a rapid and recent pop

Additional transplantation of ex vivo generated megakaryocytic cells after high-dose chemotherapy

The additional transplantation of ex vivo generated hematopoietic (post)-progenitor cells represents a possible approach to ameliorate high-dose chemotherapy induced cytopenia. We investigated the feasibility of the large-scale expansion and transplantation of autologous megakaryocytic cells in four patients with advanced solid tumors. Up to 1,460x10(6) ex vivo generated cells were administered wi

Why do hoarding birds gain fat in winter in the wrong way? Suggestions from a dynamic model

In winter, small birds should be fat to avoid starvation and lean and agile to escape predators. This means that they face a tradeoff between the costs and benefits of carrying fat reserves. Every day they must gain enough fat to survive the coming night. Food-hoarding species can afford to carry less fat than nonhoarders because they can store energy outside the body. Furthermore, hoarders should

Comparison of temperature effects on soil respiration and bacterial and fungal growth rates

Temperature is an important factor regulating microbial activity and shaping the soil microbial community. Little is known, however, on how temperature affects the most important groups of the soil microorganisms, the bacteria and the fungi, in situ. We have therefore measured the instantaneous total activity (respiration rate), bacterial activity (growth rate as thymidine incorporation rate) and

Deglacial vegetation succession and Holocene tree-limit dynamics in the Scandes Mountains, west-central Sweden: stratigraphic data compared to megafossil evidence

High-resolution records of plant macrofossils, magnetic susceptibility, and total carbon content, complemented by pollen data, were obtained from a postglacial lake sediment sequence at alpine Lake Stentjarn (987 m a.s.l.), in west-central Sweden. Holocene vegetational and environmental changes were reconstructed from the data, with particular emphasis on the deglacial establishment of terrestrial

Den första månaden efter förlossningen. Kvinnor och män som föräldrar efter barnets födelse

Studiens syfte var att undersöka hur många föräldrar som efter förlossningen önskade kontinuitet mellan graviditet och nyföddhetsperiod genom ett hembesök av den barnmorska som man lärt känna under graviditeten, hur dessa kvinnor och män formulerade sina erfarenheter och behov, om de skilde sig från föräldrar som inte önskade hembesök och om resultatet kunde ge synpunkter på en individualiserad hä

Organised Chaos: Water and Sanitation Systems in Housing Areas in Dar es Salaam, Tanzania

This thesis focuses on the provision and management of water supply and sanitation in housing areas in the City of Dar es Salaam. The main objective of the study is to contribute to knowledge and understanding of provision and management of urban water supply and sanitation in Tanzania in the context of sustainable urbanisation. A sectoral and single problem issue study approach was avoided and in

Auditory temporal grouping in newborn infants

Adults normally perceive auditory scenes in terms of sound patterns emitted by concurrently active sources. Thus pattern formation is an important process of auditory object perception. The aim of the present study was to determine whether neonates group sounds by repeating pitch patterns. Standard ("S"; p = 80%) and deviant tones ("D", p = 20%) differing only in pitch were delivered either in a r

Neural Progenitor Cells in malignancy and injury of the brain. A Trojan Horse fro gliomas?

Popular Abstract in Swedish Gliom är en familj av hjärntumörer som är graderade beroende på hur snabbt och på vilket sätt de växer. Grad IV även kallad glioblastoma multiforme, är den mest elakartade av dessa. Patienter har en medelöverlevnadsprognos på ca 12 månader med behandling, såsom kirurgi, kemo- och strålningsterapi. Tumörerna är heterogena, vilket innebär att celler har flera typer av mutGliomas are neoplasms arising in the central nervous system (CNS), and constitute the most common primary brain tumor. They are classified depending on the grade and morphology, from grade I, pilocytic astrocytoma, to grade IV, glioblastoma multiforme (GBM). Glioblastomas constitute a great challenge in treatment due to their disseminating nature. Classical treatments such as surgery, radiation th