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Defects in Axonal Transport in Inherited Neuropathies

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This list is provided as a service to those wishing to interview someone or invite someone to speak—besides Elaine Aron, who is often unavailable—on the subject of high sensitivity. Its scientific name is sensory processing sensitivity; it is not related to Sensory Processing Disorder. Each has their own specialties a selection of these are highlighted in italics and some have written books on those specialties also in italics. A few others listed are active researchers.

Because those listed below will be kept current by Elaine, they are your best resource. Bianca P. New York University; Ph. She has worked on differentiating sensitivity from clinical issues such as Autism and Schizophrenia.

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Axonal transport is a highly complex process essential for sustaining proper neuronal functioning. Disturbances can result in an altered neuronal homeostasis, aggregation of cargoes, and ultimately a dying-back degeneration of neurons. The impact of dysfunction in axonal transport is shown by genetic defects in key proteins causing a broad spectrum of neurodegenerative diseases, including inherited peripheral neuropathies. In this review, we provide an overview of the cytoskeletal components, molecular motors and adaptor proteins involved in axonal transport mechanisms and their implication in neuronal functioning.

In addition, we discuss the involvement of axonal transport dysfunction in neurodegenerative diseases with a particular focus on inherited peripheral neuropathies. Lastly, we address some recent scientific advances most notably in therapeutic strategies employed in the area of axonal transport, patient-derived iPSC models, in vivo animal models, antisense-oligonucleotide treatments, and novel chemical compounds.

Axonal transport is a highly complex process allowing movement of molecules and organelles within neurons over tremendous distances towards presynaptic nerve termini anterograde transport , and transferring material from the periphery back to the neuronal soma retrograde transport. This intracellular trafficking is crucial for sustaining proper neuronal functioning as well as clearing misfolded proteins or damaged organelles to avoid accumulation of harmful aggregates.

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