NF- B-Related Genetic Diseases

This book presents the diverse clinical, cellular and molecular manifestations of NF-KB-related genetic diseases. It shows that studying patient-related pathologies affecting the components of the NF-KB signaling pathway offers the opportunity to understand the various functions of NF-KB in humans, complementing studies performed with mouse models. In addition, people treating those patients acquire a deeper understanding of the molecular basis of the pathophysiological processes.

Autorentext
Gilles Courtois (INSERM) and colleagues are performing genetic research at the Institut des Neurosciences de Grenoble. They have demonstrated details of the NF-kB signaling pathway and collaborate on publications about Incontinentia Pigmenti, a genetic disease of the skin, hair, teeth and central nervous system. He and his team are working with the group of Annie Andrieux and Eric Denarier on mice lacking the NEMO protein, one component of the signal cascade, exclusively in their nervous system or throughout their body. The studies focus on neurological problems but will provide insight into the signaling pathway from different angles.

Inhalt
1) The NF-kB signaling pathways: players and Functions.- 2) Genetic Diseases Affecting the Canonical Pathway of NF-kB Activation.- 3) Genetic Diseases Affecting hte Non-Camonical Pathway of NF-kB Activation.- 4) Genetic Diseases Affecting Ubiquitination Processes in NF-kB Signaling.- 5)Lessons Learned from Studying NF-kB Related Genetic Diseases.