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.
This book offers a comprehensive and updated discussion of fatigue, stress corrosion and corrosion-fatigue, using many high quality figures. Stress corrosion and corrosion-fatigue are treated both from the electrochemical and fracture mechanics standpoints. This textbook, suitable for students, researchers and engineers, gathers the experience of more than 20 years of teaching fracture mechanics, fatigue and corrosion to professional engineers and running experimental tests and verifications to solve practical problems in engineering applications. As such, it is a comprehensive blend of fundamental knowledge and technical tools to address the issues of fatigue and corrosion. The book initiates with a systematic description of fatigue from a phenomenological point of view, since the early signs of submicroscopic damage in few surface grains and continues describing, step by step, how these precursors develop to become mechanically small cracks and, eventually, macrocracks whose growth is governed by fracture mechanics. But fracture mechanics is also introduced to analyze stress corrosion and corrosion assisted fatigue in a rather advanced fashion. The author dedicates a particular attention to corrosion starting with an electrochemical treatment that mechanical engineers with a rather limited knowledge of electrochemistry will well digest without any pain. The electrochemical introduction is considered an essential requirement to the full understanding of corrosion that is essentially an electrochemical process. All stress corrosion aspects are treated, from the generalized film rupture-anodic dissolution process that is the base of any corrosion mechanism to the aggression occurring in either mechanically or thermally sensitized alloys up to the universe of hydrogen embrittlement, which is described in all its possible modes of appearance. Multiaxial fatigue and out-of-phase loading conditions are treated in a rather comprehensive manner together with damage progression and accumulation that are not linear processes. Load spectra are analyzed also in the frequency domain using the Fourier transform in a rather elegant fashion full of applications that are generally not considered at all in fatigue textbooks, yet they deserve a special place and attention. The issue of fatigue cannot be treated without a probabilistic approach unless the designer accepts the shame of one-out-of-two pieces failure. The reader is fully introduced to the most promising and advanced analytical tools that do not require a normal or lognormal distribution of the experimental data, which is the most common case in fatigue. But the probabilistic approach is also used to introduce the fundamental issue of process volume that is the base of any engineering application of fatigue, from the probability of failure to the notch effect, from the metallurgical variability and size effect to the load type effect. Fractography plays a fundamental role in the post mortem analysis of fatigue and corrosion failures since it can unveil the mystery encrypted in any failure.
Die Titelstory des Sonic Seducer Jahresrückblicks 2017 ist Leaves Eyes gewidmet, der Co-Titel steht ganz im Zeichen von Visions Of Atlantis. Highlight der Ausgabe ist einerseits die exklusive EP Across The Sea zum Album Sign Of The Dragonhead von Leaves Eyes, andererseits der zweite Teil des offiziellen M´Era Luna Films 2017, moderiert von Dr. Mark Benecke. Mit DVD-Box für beide Teile des Films! Der Film umfasst über 60 Clips, darunter exklusives Livematerial, Interviews, ein 60-minütiges Hörbuchspecial zu Lovecraft Letters I und abwechslungsreiches Bonusmaterial wie z.B. eine Whiskyprobe mit Subway To Sally! Teil 1 des M´Era Luna Films 2017 ist zusammen mit der Sonic Seducer Doppelausgabe 12-2017/01-2018 erhältlich. Artists 2nd Face, Aeverium, AFI, Alphaville, ASP, Avatarium, The Beauty Of Gemina, Blutengel, Cellar Darling, Centhron, Cephalgy, Cigarettes After Sex, Clan Of Xymox, Cradle Of Filth, The Crüxshadows, Deine Lakaien, Depeche Mode, Diary Of Dreams, Dive, Dool, Dunkelschön, Edenbridge, The Eden House, Eden weint im Grab, Editors, Eisbrecher, Elane, Empathy Test, Evanescence, Exit Eden, Extramensch, Faey, Fiona, Fix8:Sëd8, Funker Vogt, Godex, Gothminister, Holygram, In Extremo, Jyrki 69, Kant Kino, Kaunan, Klez.e, KMFDM, Lacrimosa, Lea Porcelain, Leaves Eyes, Les Discrets, Letzte Instanz, Lord Of The Lost, MajorVoice, Megaherz, Merciful Nuns, M.I.N.E, Mono Inc., Mortiis, Motionless In White, Mr. Hurley & Die Pulveraffen, Myrkur, Nachtgeschrei, NamNamBulu, Nine Inch Nails, NordarR, Gary Numan, Oberer Totpunkt, Oomph!, The Other, Palast, Pale Waves, Pharmakon, The Pussybats, Randolph s Grin, Rammstein, Nicole Sabouné, Saint Astray, Saltatio Mortis, Scheuber, [:SITD:], Slowdive, Soror Dolorosa, SPECTRA*paris, Stahlmann, Subway To Sally, Suicide Commando, SynthAttack, Then Comes Silence, T.O.Y., Umbra Et Imago, Versengold, Visions Of Atlantis, VNV Nation, Welle: Erdball, Chelsea Wolfe, Xandria, X Marks The Pedwalk, X-O Planet, Ye Banished Privateers, Zola Jesus, Zynic Stage HIM, Marilyn Manson, OMD Specials & Trends 2017 Comebacks, 10 Facts, Newcomer, Rückblick der Redaktion, Soundcheck-Ranking 2017 Exklusive EP von Leaves´ Eyes 01. Across The Sea 02. Across The Sea (Sonic Acoustic Version) 03. Across The Sea (Instrumental Version) M´Era Luna 2017 Der Film, Teil 2 Live-Clips Schandmaul - Bunt und nicht braun Leæther Strip - Body Machine Body Darkhaus - Hour Of Need Feuerschwanz - Sex is Muss NamNamBulu - Memories Ost+Front - Sternenkinder + weitere Videoclips Interviews Solar Fake Blutengel Covenant Megaherz White Lies Unzucht DAF The Crüxshadows Bonusmaterial M´Era Luna 2017 Whiskytasting mit Subway To Sally Gothic Fashion Show Impressionen / Autogrammstunden Backstage mit Dr. Mark Benecke Bonusmaterial Evanescence - Interview Leaves Eyes - Interview Roterfeld - Interview Umbra Et Imago - Interview Hörbuchspecial: Hörbuch-Ausschnitt Lovecraft Letters