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The hygrothermal behaviour of an outdoor ventilated crawl space with two different designs of the floor structure was investigated. The first design had 250 mm insulation and visible wooden beams towards the crawl space. The second design had 300 mm insulation and no visible wooden beams. One year of measurements was compared with simulations of temperature and moisture condition in the floor structure and crawl space. The measurements showed that the extra 50 mm insulation placed below the beams reduced moisture content in the beams below 20 weight% all year. A reasonable agreement between the measurements and simulations was found; however, the evaporation from the soil was a dominant parameter affecting the hygrothermal response in the crawl space and floor structure.

Internal insulation is used in many heritage buildings to ensure a better indoor environment and reduce energy use. This article describes measurement results from installing an internal insulation system in a Danish multi-story building. The internal insulation system consists of a fully glued vapour tight insulated plasterboard. To reduce the risk for wood decay and mould growth at the wooden beam ends in the floor construction, a 100-200 mm uninsulated gap was left above and below the floor construction. Measurements include the effect of orientation, degree of façade repair and influence of indoor moisture content. Results show in general acceptable hygrothermal conditions behind the insulation system. However, a gradual increase in relative humidity at sun-exposed walls was registered due to ‘summer condensation’. The degree of surface repair also showed an influence on relative humidity levels during this period. At the uninsulated gap above the floor, a higher moisture content indoor resulted in a higher relative humidity, though without exceeding the threshold for mould growth. However, a large temperature range was registered. The influence of the uninsulated gap on reducing the risk of wood decay and mould growth at the beam ends could therefore not unambiguously be confirmed.

In the design of very well-insulated homes, there is a need for a more nuanced design that takes into account winter and summer conditions. In this paper, we compare a traditional design for a typical Danish single-family house with large glazing areas oriented towards the south and smaller glazing areas towards the north, and a design with an even window distribution where the glazing-to-floor ratio is the same for each room. We found that the use of solar gains through south-oriented windows is not as important as is traditionally believed because, in well-insulated homes, space heating demand is not reduced much by having larger south-facing windows. Furthermore, we found that there is a g-value above which the additional solar gains through south-oriented windows do not help reduce space heating demand, and it becomes important to use solar shading or glazing with solar-control coating as a cheaper alternative to reduce overheating. Maximum window sizes from an overheating perspective were identified that are larger than the optimal window sizes for space heating demand. However, we show that the difference in space heating demand with optimal window size and with larger window sizes is small, so it is up to the building owner to decide whether or not he wants larger glazing areas to allow for more daylight. And windows can be positioned in the facade with considerable architectural freedom. However, we do recommend an even distribution of the glazing-to-floor ratio, because this generally provides an improved thermal indoor environment in south-oriented rooms and will ensure a better daylight level especially in north-oriented rooms. We also show that the optimal window size is influenced by thermal zone configuration and that there is a need for models in which a difference is made between zones with direct and with non-direct solar gains.

Basonuclins 1 and 2 are nuclear proteins specific to vertebrates. They have recently been shown to be the orthologs of the DISCO proteins of insects. All of these proteins have an essential function in embryonic development. It is likely that they are transcription factors with multiple gene targets, some of which are transcribed by DNA polymerase I, and others by polymerase II. It remains to be found which targets are common to the two basonuclins and even to the DISCO proteins, and why certain cell types possess either basonuclin 1 or basonuclin 2, while others possess both. The implication of the basonuclins in several human diseases adds to their interest as regulatory proteins.

The number of mRNAs and proteins that can be produced from a single gene is known to be increased by the number of start sites and by multiple splicing of products. A few genes have been found to generate extraordinarily large numbers of splicing isoforms. In the human, the largest number, nearly 2000 mRNA isoforms, has been reported for the neurexin 3α gene. However, the biological significance of alternative splicing often remains unclear because many alternative transcripts contain early translational stops and are thought to be rapidly degraded. We demonstrate here that human basonuclin 2 (bn2; approved gene symbol BNC2) transcripts are initiated from six promoters, are alternatively spliced at multiple positions, and are polyadenylated at four sites. Characterization of nearly 100 bn2 mRNA isoforms suggests that each promoter, splice site, and poly(A) addition site is used independently. The bn2 gene has therefore the potential to generate up to 90,000 mRNA isoforms encoding more than 2000 different proteins. Because alternative exons affect the position of the first methionine codon, the length of the coding region, and the position of the translational stop, the encoded proteins range in size from 43 to 1211 amino acids and some bear no sequence similarity to others. PCR analysis and transient expression in HeLa cells show that the major bn2 mRNA isoforms are stable and are translated into equally stable proteins, even when the mRNA bears an early translational stop.

BNC2 is an extremely conserved zinc finger protein with important functions in the development of craniofacial bones and male germ cells. Because disruption of the Bnc2 gene in mice causes neonatal lethality, the function of the protein in adult animals has not been studied. Until now BNC2 was considered to have a wider tissue distribution than its paralog, BNC1, but the precise cell types expressing Bnc2 are largely unknown. We identify here the cell types containing BNC2 in the mouse and we show the unexpected presence of BNC1 in many BNC2-containing cells. BNC1 and BNC2 are colocalized in male and female germ cells, ovarian epithelial cells, sensory neurons, hair follicle keratinocytes and connective cells of organ capsules. In many cell lineages, the two basonuclins appear and disappear synchronously. Within the male germ cell lineage, BNC1 and BNC2 are found in prospermatogonia and undifferentiated spermatogonia, and disappear abruptly from differentiating spermatogonia. During oogenesis, the two basonuclins accumulate specifically in maturing oocytes. During the development of hair follicles, BNC1 and BNC2 concentrate in the primary hair germs. As follicle morphogenesis proceeds, cells possessing BNC1 and BNC2 invade the dermis and surround the papilla. During anagen, BNC1 and BNC2 are largely restricted to the basal layer of the outer root sheath and the matrix. During catagen, the compartment of cells possessing BNC1 and BNC2 regresses, and in telogen, the two basonuclins are confined to the secondary hair germ. During the next anagen, the BNC1/BNC2-containing cell population regenerates the hair follicle. By examining Bnc2−/− mice that have escaped the neonatal lethality usually associated with lack of BNC2, we demonstrate that BNC2 possesses important functions in many of the cell types where it resides. Hair follicles of postnatal Bnc2−/− mice do not fully develop during the first cycle and thereafter remain blocked in telogen. It is concluded that the presence of BNC2 in the secondary hair germ is required to regenerate the transient segment of the follicle. Postnatal Bnc2−/− mice also show severe dwarfism, defects in oogenesis and alterations of palatal rugae. Although the two basonuclins possess very similar zinc fingers and are largely coexpressed, BNC1 cannot substitute for BNC2. This is shown incontrovertibly in knockin mice expressing Bnc1 instead of Bnc2 as these mice invariably die at birth with craniofacial abnormalities undistinguishable from those of Bnc2−/− mice. The function of the basonuclins in the secondary hair germ is of particular interest.

Absence of mitosis and meiosis are distinguishing properties of male germ cells during late fetal and early neonatal periods. Repressors of male germ cell meiosis have been identified, but mitotic repressors are largely unknown, and no protein repressing both meiosis and mitosis is known. We demonstrate here that the zinc-finger protein BNC2 is present in male but not in female germ cells. In testis, BNC2 exists as several spliced isoforms and presumably binds to DNA. Within the male germ cell lineage, BNC2 is restricted to prospermatogonia and undifferentiated spermatogonia. Fetal prospermatogonia that lack BNC2 multiply excessively on embryonic day (E)14.5 and reenter the cell cycle prematurely. Mutant prospermatogonia also engage in abnormal meiosis; on E17.5, Bnc2−/− prospermatogonia start synthesizing the synaptonemal protein SYCP3, and by the time of birth, many Bnc2−/− prospermatogonia have accumulated large amounts of nonfilamentous SYCP3, thus appearing to be blocked at leptonema. Bnc2−/− prospermatogonia do not undergo proper male differentiation, as they lack almost all the mRNA for the male-specific methylation protein DNMT3L and have increased levels of mRNAs that encode meiotic proteins, including STRA8. Bnc2−/− prospermatogonia can produce spermatogonia, but these enter meiosis prematurely and undergo massive apoptotic death during meiotic prophase. This study identifies BNC2 as a major regulator of male germ stem cells, which is required for repression of meiosis and mitosis in prospermatogonia, and for meiosis progression during spermatogenesis. In view of the extreme evolutionary conservation of BNC2, the findings described here are likely to apply to many species.

There is a lack of international research focusing on how the siblings-in-law of adults with a learning disability experience their commitment to provide support. Using narrative research, the authors have aimed to gain an insight into the perspectives of 14 siblings-in-law (living in the Flemish part of Belgium) about the decision to live together with a family member who has a learning disability. The decision to live together usually begins with a crisis. However, the decision process is a complex mechanism; it involves dynamics and events within a family (between family members both with and without blood relationships) and is intensified by experiences within a society that holds different views about families and disability. We are aided in gaining a deeper understanding of this complex process by considering interpretations based on the contextual framework of Boszormenyi-Nagy.

We studied a man with distal hypospadias, partial anomalous pulmonary venous return, mild limb-length inequality and a balanced translocation involving chromosomes 9 and 13. To gain insight into the etiology of his birth defects, we mapped the translocation breakpoints by high-resolution comparative genomic hybridization (CGH), using chromosome 9- and 13-specific tiling arrays to analyze genetic material from a spontaneously aborted fetus with unbalanced segregation of the translocation. The chromosome 13 breakpoint was ∼400 kb away from the nearest gene, but the chromosome 9 breakpoint fell within an intron of Basonuclin 2 (BNC2), a gene that encodes an evolutionarily conserved nuclear zinc-finger protein. The BNC2/Bnc2 gene is abundantly expressed in developing mouse and human periurethral tissues. In all, 6 of 48 unrelated subjects with distal hypospadias had nine novel nonsynonymous substitutions in BNC2, five of which were computationally predicted to be deleterious. In comparison, two of 23 controls with normal penile urethra morphology, each had a novel nonsynonymous substitution in BNC2, one of which was predicted to be deleterious. Bnc2(-/-) mice of both sexes displayed a high frequency of distal urethral defects; heterozygotes showed similar defects with reduced penetrance. The association of BNC2 disruption with distal urethral defects and the gene's expression pattern indicate that it functions in urethral development.

Retinoic acid-related Orphan Receptor alpha (RORα; NR1F1) is a widely distributed nuclear receptor involved in several (patho)physiological functions including lipid metabolism, inflammation, angiogenesis, and circadian rhythm. To better understand the role of this nuclear receptor in liver, we aimed at displaying genes controlled by RORα in liver cells by generating HepG2 human hepatoma cells stably over-expressing RORα. Genes whose expression was altered in these cells versus control cells were displayed using micro-arrays followed by qRT-PCR analysis. Expression of these genes was also altered in cells in which RORα was transiently over-expressed after adenoviral infection. A number of the genes found were involved in known pathways controlled by RORα, for instance LPA, NR1D2 and ADIPOQ in lipid metabolism, ADIPOQ and PLG in inflammation, PLG in fibrinolysis and NR1D2 and NR1D1 in circadian rhythm. This study also revealed that genes such as G6PC, involved in glucose homeostasis, and AGRP, involved in the control of body weight, are also controlled by RORα. Lastly, SPARC, involved in cell growth and adhesion, and associated with liver carcinogenesis, was up-regulated by RORα. SPARC was found to be a new putative RORα target gene since it possesses, in its promoter, a functional RORE as evidenced by EMSAs and transfection experiments. Most of the other genes that we found regulated by RORα also contained putative ROREs in their regulatory regions. Chromatin immunoprecipitation (ChIP) confirmed that the ROREs present in the SPARC, PLG, G6PC, NR1D2 and AGRP genes were occupied by RORα in HepG2 cells. Therefore these genes must now be considered as direct RORα targets. Our results open new routes on the roles of RORα in glucose metabolism and carcinogenesis within cells of hepatic origin.

Basonuclin 1 and the recently discovered basonuclin 2 are vertebrate proteins with multiple paired C(2)H(2) zinc fingers. It has long been known that the zinc fingers of basonuclin 1 closely resembled those of the Drosophila disconnected and discorelated proteins, two proteins essential for head development, but the relation between the basonuclins and the disco proteins has remained unclear because the putative function of basonuclin 1 in the control of keratinocyte growth potential appeared unrelated to that of disco and there was no resemblance between basonuclin 1 and Drosophila disco outside of the zinc fingers. The recent generation of a basonuclin-2 knockout has demonstrated that basonuclin 2 shares with disco a function in head development and the availability of new arthropod genome sequences has shown that the basonuclins are the vertebrate orthologs of the insect disco proteins. All these proteins are thought to be transcription factors, and it will have to be determined to what extent they share similar targets.

Basonuclin 2 is a recently discovered zinc finger protein of unknown function. Its paralog, basonuclin 1, is associated with the ability of keratinocytes to multiply. The basonuclin zinc fingers are closely related to those of the Drosophila proteins disco and discorelated, but the relation between disco proteins and basonuclins has remained elusive because the function of the disco proteins in larval head development seems to have no relation to that of basonuclin 1 and because the amino acid sequence of disco, apart from the zinc fingers, also has no similarity to that of the basonuclins. We have generated mice lacking basonuclin 2. These mice die within 24 h of birth with a cleft palate and abnormalities of craniofacial bones and tongue. In the embryonic head, expression of the basonuclin 2 gene is restricted to mesenchymal cells in the palate, at the periphery of the tongue, and in the mesenchymal sheaths that surround the brain and the osteocartilagineous structures. In late embryos, the rate of multiplication of these mesenchymal cells is greatly diminished. Therefore, basonuclin 2 is essential for the multiplication of craniofacial mesenchymal cells during embryogenesis. Non- Drosophila insect databases available since 2008 reveal that the basonuclins and the disco proteins share much more extensive sequence and gene structure similarity than noted when only Drosophila sequences were examined. We conclude that basonuclin 2 is both structurally and functionally the vertebrate ortholog of the disco proteins. We also note the possibility that some human craniofacial abnormalities are due to a lack of basonuclin 2.

Diseases of polyglutamine expansion, Alzheimer's disease and Parkinson's disease are neurodegenerative diseases associated with insoluble protein aggregates and neuronal death. These diseases constitute a group of devastating diseases for which there is currently little treatment. The protein aggregates may be the cause of neuronal death, although there is some controversy as to which form of aggregation (oligomers, polymers or microscopic aggregates) is the most toxic. More than a decade ago, the participation of transglutaminases in the formation of the abnormal protein aggregates was proposed. Transglutaminases are a large family of enzymes that catalyze the formation of N(sigma) (gamma-glutamyl)-lysine isodipeptide crosslinks between proteins. In this review, we summarize the evidence supporting the participation of transglutaminase in diseases of the central nervous system. We also describe newly developed transglutaminase inhibitors and their potential use as therapeutic agents in neurological disease.

The epidermis of birds differs from that of mammals by the presence of intracellular lipid droplets and the absence of sebaceous glands. We describe here the cultivation of chicken epidermal keratinocytes; these cells cannot be grown in medium supplemented with the usual fetal bovine serum even in the presence of supporting 3T3 cells, but they can grow from single cells in the presence of supporting 3T3 cells and 10% chicken serum. As revealed by their cell structure, their protein composition, and their gene expression, chicken keratinocytes possess the general properties of mammalian keratinocytes. They ultimately undergo in culture a process of terminal differentiation in which their nucleus is destroyed and a cornified envelope is formed. Chicken keratinocytes also show important properties that mammalian keratinocytes do not possess: they accumulate neutral lipids, usually in the form of a single perinuclear droplet; they accumulate carotenoids; they synthesize beta-keratins; and their multiplication requires a non-lipid factor, present in chicken serum but not in fetal calf serum. The lipid-synthesizing function of sebocytes in mammals is carried out by the keratinocytes themselves in birds. The availability of cultured chicken keratinocytes should allow studies that were hitherto impossible such as the tracing of the keratinocyte lineage during development of the chicken embryo and the investigation of the complete life cycle of viruses that require specific chicken keratinocyte products (such as Marek's disease virus).

Basonuclin is a zinc finger protein specific to basal keratinocytes and germ cells. In keratinocytes, basonuclin behaves as a stem cell marker and is thought to be a transcription factor that maintains proliferative capacity and prevents terminal differentiation. The human gene is located on chromosome 15. We have discovered in the chicken the existence of basonuclin 2, a basonuclin homolog. We also report the entire sequence of mouse and human basonuclin 2; the corresponding genes are located on mouse chromosome 4 and human chromosome 9. Although the amino acid sequence of basonuclin 2 differs extensively from that of basonuclin 1, the two proteins share essential features. Both contain three paired zinc fingers, a nuclear localization signal, and a serine stripe. The basonuclin 2 mRNA has a wider tissue distribution than the basonuclin 1 mRNA: it is particularly abundant in testis, kidney, uterus, and intestine. The extreme conservation of the basonuclin 2 amino acid sequence across vertebrates suggests that basonuclin 2 serves an important function, presumably as a regulatory protein of DNA transcription.

SBi-anvisning 265 omhandler generelle krav og anbefalinger til vådrum samt vand- og afløbsinstallationer i nybyggede småhuse. Vådrum kan være baderum, bryggers og andre rum, hvor der forventes påvirkning af vand eller høj relativ luftfugtighed.Løsningerne i anvisningen opfylder bestemmelserne i Bygningsreglement 2015 (BR15).Anvisningen indgår i en serie af SBi-anvisninger om småhuse. Småhuse omfatter fritliggende og sammenbyggede enfamiliehuse med lodret lejlighedsskel i indtil to etager samt eventuel kælder. Det omfatter også kæde- og rækkehuse, samt sommerhuse.Målgruppen for anvisningen er projekterende arkitekter, ingeniører samt udførende og tilsynsførende. Anvisningen kan desuden anvendes af den kommende bygningsejer og som lærebog ved byggetekniske uddannelser.

In the Nordic Innovation Centre Project, SuccessFamilies, the main objective is to change the business environment in order to speed up the implementation of sustainable renovation of single-family houses – proposing new service concepts that will combine both the technical solutions, financing services as well as other promoting issues to overcome the behavioural, organizational, legal and social barriers that exist in sustainable renovation. A starting point for such a change has been to get an overview of the existing sustainable renovation concepts, i.e. full-service concepts and technical concepts. The paper describes a few Nordic examples of full-service models for renovation of single-family houses which entered the market recently and the provisional success of these concepts. The existing Nordic single-family house renovation market is dominated by a craftsman based approach with individual solutions, traditional warehouses ”do-it-yourself-shops” and some actors marketing single products. To speed up the implementation of sustainable renovation of single-family houses there is a great need for full-service packages. Existing technical renovation concepts, typically focusing on application of only a few of the available technical solutions, have not been successful in realizing large scale energy efficiency gains.

Within an ERANET-ERACOBUILD project, this study investigates the opportunities and barriers to establish a “one stop shop” with an integrated supply side, to counteract the fragmented offer in sustainable renovation of single-family houses and to increase the level of knowledge, skills and innovations. Aspects of providing reliable information and guidance for house-owners are also included. Starting from a survey directed to the Flemish construction sector, we tried to distill the viewpoint and willingness of enterprises to cooperate in such a one-stop-shop idea. The survey concludes that the increase of holistic very low energy renovations is expected in a short time and that a large percentage of companies is willing to work together. However, socio-technical and know-how barriers to accomplish this still remain. With these results - and an analysis of existing websites providing an interface between suppliers and owner-occupants - business models, training and other activities will be set up to establish a one-stop shop model within the continuation of this project. A test case is foreseen.