Longchamp serial number meaning. Background The project is organised as collaboration between Energinet and the polish gas transmission system operator GAZ-SYSTEM S.A. Baltic Pipe will include expansion of the Polish and Danish gas infrastructure and will begin gas transmission services by October 2022. The new pipeline will expand the gas transmission capacity by up to 10 billion cubic metres of gas per year.

In comparison, the total Danish gas consumption for 2016 was 2.5 billion cubic metres. Win-win for both Denmark and Poland A feasibility study has shown that the Baltic Pipe Project can bring significant socioeconomic benefits to Poland, Denmark and the Baltic and Central & Eastern European regions. Important European Project EU has included Baltic Pipe on its list of key infrastructure projects that are of common interest to Europe – also known as “PCI projects”. This is due to the essential role Baltic Pipe could play in contributing to the development of Europe’s internal market for gas, and the fortification of EU’s security of supply. Investment Decision The final investment decision on Baltic Pipe was made in November 2018. As a precondition for greenlighting Baltic Pipe, Energinet and GAZ-SYSTEM S.A. Launched an “Open Season” process in the second half of 2017, which invited market players to submit binding bids for capacity in the projected pipeline. The result of the Open Seasom was positive Technical description Baltic Pipe will include the following technical systems and installation: • A 105-110 kilometre long, new offshore gas pipeline from Norway’s pipeline Europipe II in the North Sea to a receiving terminal.

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• Expansion of the Danish transmission system with a new gas pipeline, approximately 210 kilometres long. • A compressor station in Zealand.

The compressor station increases the pressure of the gas in the pipeline in the Baltic Sea. • A 260-310 kilometre long offshore gas pipeline in the Baltic Sea between Denmark and Poland.

GAZ-SYSTEM is in charge of establishing a gas pipeline across the Baltic Sea between Denmark and Poland. • Expansions of Poland’s transmission system. Energinet is in charge of establishing the first three components, while GAZ-SYSTEM is responsible for the pipeline in the Baltic Sea and expansions in Poland. Costs The total cost estimate for Baltic Pipe is 12-16 billion kroners.The costs are split equally between Energinet and GAZ-SYSTEM.

Abstract Single gene disorders with Mendelian inheritance patterns have contributed greatly to the identification of genes and pathways implicated in genetic disease. In these cases, molecular analysis predicts disease status relatively directly. However, there are many abnormalities which show familial recurrence and have a clear genetic component, but do not show regular Mendelian segregation patterns. Defining the causative gene for non-Mendelian diseases is more difficult, and even when the underlying gene is known, there is uncertainty for prenatal prediction.

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However, detailed examination of the different mechanisms that underlie non-Mendelian segregation provides insight into the types of interaction that regulate more complex disease genetics. Mendelian inheritance patterns are well-established, and readily recognizable as ‘textbook’ examples, for many single gene diseases (), and a few digenic cases (–). However, in most clinical genetics settings many cases are seen where the disease diagnosed is well known to have a strong genetic component, and show some familial recurrence, but no clear Mendelian inheritance. Such cases clearly pose additional problems in counselling and the estimation of recurrence risk. Here, we review some of the different molecular mechanisms that lead to such irregular inheritance patterns, focussing mostly on diseases where at least one implicated gene and some underlying mutations have been identified.

It is useful to attempt to categorize the different ways in which the observed inheritance patterns are generated (Table ) and then to consider in more detail some examples in each category. Some detailed molecular mechanisms underlying non-Mendelian inheritance patterns will be unfolded below, but first some general concepts need to be clarified. Incomplete penetrance (), where not all mutation carriers present with the expected phenotype, is commonly observed in human family studies. This forms a continuum with variable expressivity, which can be so extreme that subtle manifestations of carrier status are sometimes only identified with hindsight (). Even some of the most classical Mendelian traits, like cystic fibrosis (CF), show complex variation (,). A significant proportion of variability can be ascribed to allelic differences (,), some of which will be cryptic regulatory variation, influencing gene expression levels (,). The existence of widespread variation is not surprising, as gene products fulfil their function through finely tuned interactions with other cellular components, often showing some degree of threshold requirement ().