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1.
  • Ström, Anna-Lena, 1975-, et al. (författare)
  • Axonal transport and motor neuron disease
  • 2011
  • Ingår i: Cytoskeleton of the Nervous System. - New York : Springer-Verlag New York. - 9781441967862 ; , s. 529-544
  • Bokkapitel (refereegranskat)abstract
    • Describes cytoskeleton in axonal development and pathology, microtubules and associated proteins, neurofilaments and interacting proteins, actin and its binding proteins, and glial fibrillary acidic protein 2. Focuses on functional significance of neuronal cytoskeleton in axonal transport 3. Encourages further development of unifying principles, stimulates new conceptual and technical approaches toward a better understanding of cytoskeleton functions in health and disease Without a cytoskeleton, a neuron or glial cell would be a shapeless jelly mass unable to function in the milieu of the brain. If we are to understand neuronal cells function in health and disease, we must determine how the cytoskeleton forms and contributes to neural physiology and pathobiology. Cytoskeleton of the Nervous System provides a comprehensive, authoritative and up-to-date account of what we now know and what we want to know in the near future--about the functioning of the cytoskeleton of neuronal cells at the molecular level. In lively accounts, which are unafraid to address controversy, Cytoskeleton of the Nervous System introduces readers to the most sophisticated concepts and latest discoveries: from overexpression systems to knock-out models for specific cytoskeletal proteins, from continuous transport assays in vivo to live-cell imaging in primary neurons, and from factors regulating cytoskeleton behavior to the dysregulation of these processes leading to neurological disease.
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2.
  • Ström, Anna-Lena, et al. (författare)
  • Retrograde axonal transport and motor neuron disease
  • 2008
  • Ingår i: Journal of Neurochemistry. - : Wiley. - 0022-3042 .- 1471-4159. ; 106:2, s. 495-505
  • Tidskriftsartikel (refereegranskat)abstract
    • Transport of material between extensive neuronal processes and the cell body is crucial for neuronal function and survival. Growing evidence shows that deficits in axonal transport contribute to the pathogenesis of multiple neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Here we review recent data indicating that defects in dynein-mediated retrograde axonal transport are involved in ALS etiology. We discuss how mutant copper-zinc superoxide dismutase (SOD1) and an aberrant interaction between mutant SOD1 and dynein could perturb retrograde transport of neurotrophic factors and mitochondria. A possible contribution of axonal transport to the aggregation and degradation processes of mutant SOD1 is also reviewed. We further consider how the interference with axonal transport and protein turnover by mutant SOD1 could influence the function and viability of motor neurons in ALS.
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