Choose an application

• Reference Manager
• EndNote
• RefWorks (Direct export to RefWorks)

The formation of the proper pattern of neuronal circuits during development is critical for the normal function of the vertebrate brain and for the survival of the organism. Circuit tracing studies spanning the past 100 years have revealed the beauty and exquisite intricacy of this pattern, which represents the most complex biological system known. In humans, aberrant circuit formation is a likely underlying cause of a wide variety of birth defects and neurological disorders, including autism, intellectual disability, and schizophrenia. Furthermore, future therapeutic approaches to restoring the function of damaged neural circuits will require a better understanding of the developmental constraints under which those circuits were originally assembled. For these reasons, elucidating the molecular mechanisms of neural circuit formation is a major goal of neurobiology today.Substantial progress towards this goal has been made over the past decade, and the pace of research in the field continues to accelerate with the development of novel molecular techniques and a wider variety of genetic model systems, including zebrafish and nematodes in addition to fruit flies and mice. The aim of this Research Topic is to bring together the many strands of research that shed light on the mechanisms driving neural circuit formation: studies of the differentiation of distinct neuronal subtypes; the formation of dendritic arbors and the elaboration of postsynaptic spines; the pathfinding, targeting, and branching of axons; the proper apposition of specific pre- and post-synaptic terminals; the emerging role of glial cells in facilitating synaptogenesis and synapse elimination; and the mutations behind the aberrant circuitry that leads to neurological disorders. We seek to highlight not only newly identified molecular mechanisms, but also technical advances that have allowed progress in the field to grow exponentially, including novel imaging techniques and the proliferation of large-scale “-omics” studies. We hope that this Research Topic will provide a forum for top researchers in the field to present new data, formulate novel hypotheses and models, and critically review recent progress in each step of neural circuit formation.

Neuroscience --- Human Anatomy & Physiology --- Health & Biological Sciences --- neural circuit --- dendrite arborization --- Cell Adhesion Molecules --- axon guidance --- synapse formation

Choose an application

• Reference Manager
• EndNote
• RefWorks (Direct export to RefWorks)

Lysosomal storage disorders are a heterogenoeus group of rare genetic conditions affecting worldwide population and often exhibiting severe clinical manifestations. During the last two decades, the joined collaboration between scientists and clinicians has allowed to offer valuable therapeutic options to affected patients. Therefore, the tight connection between basic science and clinical medicine represents the gold standard approach to these disorders. In this context, the present book collects a piece of current scientific advances in the knowledge of disease pathogenesis and in the development of novel diagnostic and therapeutic strategies for some of these diseases. Altogether, these articles define and recapitulate which essential steps are required during the clinical management of a rare inherited disorder and describe forthcoming advances and a breakthrough in the field of lysosomal diseases.

mucopolysaccharidosis IIIB --- quantitative proteomics --- NAGLU --- lysosomes --- Gaucher disease --- bone involvement --- enzyme replacement therapy --- substrate reduction therapy --- Osteoimmunology --- RANK/RANKL --- Osteopontin --- MIP-1β --- mucolipidosis II --- sortilin --- TGF-beta --- cathepsin D --- Fabry disease --- alpha-galactosidase A --- endocytosis --- lysosome --- IGF2R/M6P --- clathrin --- chloroquine --- lysosomal diseases --- precision medicine --- pharmacological chaperones --- gene therapy. --- Pompe disease --- lysosomal targeting --- autophagy --- gene therapy --- muscle --- satellite cells --- rhGAA --- glycogen --- lysosomal α-glucosidase --- GAA biomarker --- Gaucher Disease --- Wnt/β-catenin --- Dkk1 --- Wnt3a --- iPSC --- neuronopathy --- Krabbe disease --- Twitcher mouse --- psychosine --- visual system --- visual cortex --- astrogliosis --- mucopolysaccharidosis type I --- Hurler syndrome --- hematopoietic stem cell transplantations --- animal models --- experimental therapies --- axon guidance --- lysosomal storage disorders --- neuronal circuit --- α-galactosidase A --- A4GALT --- globotriaosylceramide (Gb3) --- globotriaosyl-sphingosine (lysoGb3) --- pharmacological chaperone therapy --- exosomes --- endocytic pathways --- neurodegenerative disease --- Parkinson disease --- lysosomal storage disorder --- viral vectors --- newborn screening --- variant interpretation --- second tier test --- tandem mass spectrometry --- lyso-Gb3 --- dried blood spot --- GLA gene --- globotriaosylsphingosine --- biomarkers

Choose an application

• Reference Manager
• EndNote
• RefWorks (Direct export to RefWorks)

A revelatory tale of how the human brain develops, from conception to birth and beyondBy the time a baby is born, its brain is equipped with billions of intricately crafted neurons wired together through trillions of interconnections to form a compact and breathtakingly efficient supercomputer. Zero to Birth takes you on an extraordinary journey to the very edge of creation, from the moment of an egg’s fertilization through each step of a human brain’s development in the womb—and even a little beyond.As pioneering experimental neurobiologist W. A. Harris guides you through the process of how the brain is built, he takes up the biggest questions that scientists have asked about the developing brain, describing many of the thrilling discoveries that were foundational to our current understanding. He weaves in a remarkable evolutionary story that begins billions of years ago in the Proterozoic eon, when multicellular animals first emerged from single-cell organisms, and reveals how the growth of a fetal brain over nine months reflects the brain’s evolution through the ages. Our brains have much in common with those of other animals, and Harris offers an illuminating look at how comparative animal studies have been crucial to understanding what makes a human brain human.An unforgettable chronicle of one of nature’s greatest achievements, Zero to Birth describes how the brain’s incredible feat of orchestrated growth ensures that every brain is unique, and how breakthroughs at the frontiers of science are helping us to decode many traits that only reveal themselves later in life.

SCIENCE / Life Sciences / Neuroscience. --- Action potential. --- Agrin. --- Angiogenesis. --- Antibody. --- Apoptosis. --- Astrocyte. --- Axon guidance. --- Axon. --- Blastula. --- Brain asymmetry. --- Broca's area. --- Cancer cell. --- Cell type. --- Cerebral atrophy. --- Cerebral cortex. --- Charles Darwin. --- Chemical synapse. --- Critical period. --- Cyclopamine. --- Degenerative disease. --- Dendrite. --- Down syndrome. --- Ectoderm. --- Embryo. --- Embryology. --- Endocrinology. --- Eric Knudsen. --- Evolution. --- FOXP2. --- Filopodia. --- Forebrain. --- Ganglion cell. --- Gastrulation. --- Gene. --- Growth cone. --- Hans Spemann. --- Hebbian theory. --- Hindbrain. --- Hirschsprung's disease. --- Homeosis. --- Hox gene. --- Human brain. --- Immortalised cell line. --- John Gurdon. --- Lancelot Hogben. --- Lateralization of brain function. --- Marian Diamond. --- Midbrain. --- Model organism. --- Morphogen. --- Motor neuron. --- Muscle. --- Myocyte. --- Nematode. --- Nervous tissue. --- Neural crest. --- Neural development. --- Neural plate. --- Neural stem cell. --- Neural tube defect. --- Neural tube. --- Neuroblast. --- Neuroblastoma. --- Neuroepithelial cell. --- Neuroglia. --- Neuroimaging. --- Neuron doctrine. --- Neuron. --- Organoid. --- Petri dish. --- Progenitor cell. --- Proneural genes. --- Protein. --- Protocadherin. --- Purkinje cell. --- Reeler. --- Reelin. --- Renshaw cell. --- Reticular theory. --- Retinoic acid. --- Roel Nusse. --- Ross Granville Harrison. --- Sarcoma. --- Sonic hedgehog. --- Spina bifida. --- Spinal cord. --- Spindle apparatus. --- Stem cell. --- Sydney Brenner. --- Synapsis. --- Synaptic plasticity. --- Thomas Hunt Morgan. --- Thrombospondin. --- Torsten Wiesel. --- Transformation (genetics). --- Twin. --- Vertebrate. --- Visual word form area. --- White blood cell. --- Zygote. --- Brain --- Growth. --- Neuronal Plasticity --- SCIENCE / Life Sciences / Neuroscience --- SCIENCE / Life Sciences / Developmental Biology --- growth & development --- embryology --- physiology