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Neurofibromatosis Type 1
Neurofibromatosis type 1 (NF1), caused by mutational inactivation of the NF1 tumour suppressor gene, is one of the most common dominantly inherited human disorders, affecting 1 in 3000 individuals worldwide. This book presents in concise fashion, but as comprehensively as possible, our current state of knowledge on the molecular genetics, molecular biology and cellular biology of this tumour predisposition syndrome. Written by internationally recognized experts in the field, the 44 chapters that constitute this edited volume provide the reader with a broad overview of the clinical features of the disease, the structure and expression of the NF1 gene, its germ line and somatic mutational spectra and genotype-phenotype relationships, the structure and function of its protein product (neurofibromin), NF1 modifying loci, the molecular pathology of NF1-associated tumours, animal models of the disease, psycho-social aspects and future prospects for therapeutic treatment.
Advanced Communication and Control Methods for Future Smartgrids
Proliferation of distributed generation and the increased ability to monitor different parts of the electrical grid offer unprecedented opportunities for consumers and grid operators. Energy can be generated near the consumption points, which decreases transmission burdens and novel control schemes can be utilized to operate the grid closer to its limits. In other words, the same infrastructure can be used at higher capacities thanks to increased efficiency. Also, new players are integrated into this grid such as smart meters with local control capabilities, electric vehicles that can act as mobile storage devices, and smart inverters that can provide auxiliary support. To achieve stable and safe operation, it is necessary to observe and coordinate all of these components in the smartgrid.
National Union Catalog
Includes entries for maps and atlases.
Enciclopedia Internacional de Pseud·ʼnimos
This Encyclopedia is the first to compile pseudonyms from all over the world, from all ages and occupations in a single work: some 500,000 pseudonyms of roughly 270,000 people are deciphered here. Besides pseudonyms in the narrower sense, initials, nick names, order names, birth and married names etc. are included. The volumes 1 to 9 list persons by their real names in alphabetical order. To make the unequivocal identification of a person easier, year and place of birth and death are provided where available, as are profession, nationality, the pseudonym under which the person was known, and finally, the sources used. The names of professions given in the source material have been translated into English especially for this encyclopaedia. In the second part, covering the volumes 10 to 16, the pseudonyms are listed alphabetically and the real names provided. Approx. 500,000 pseudonyms of about 270,000 persons First encyclopedia including pseudonyms from all over the world, all times and all occupations Essential research tool for anyone wishing to identify persons and names for his research within one single work
F.C.I. Siza
None
Gaceta de Madrid
None
Dibujo técnico - Enciclopedia.
El aprendizaje del Dibujo Técnico ha supuesto siempre un exceso bibliográfico, cuya labor de recopilación dificultaba su concreción. Los autores han pretendido cubrir este vacío con una obra que inicie y complete el estudio del Dibujo Técnico, rigurosamente actualizado en la Normalización y desarrollado con carácter práctico. Los primeros diez temas son un resumen práctico de la teoría del Dibujo. La parte central recoge los temas de Normalización más importantes de acuerdo a las normas UNE y a las normas internacionales y la última parte consta de ejercicios prácticos a los que se ha dedicado especial interés, dado el carácter de esta obra.
