Extracerebral biopsy in lysosomal and peroxisomal disorders. Ultrastructural findings.
The lysosomal and peroxisomal disorders are characterized by specific storage affecting mainly the central nervous system with involvement of the peripheral nervous system and visceral organs.
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#1 Jun 17, 2013
Extracerebral biopsy in lysosomal and peroxisomal disorders can be biopsed from the mouth tissue' it is problem of incorrectly signalling by calcium channels affecting neurotrophin (NT)which is a family of growth factors' is the most widely studied of the extracellular signaling influence of neuronal development and guidance. neurotrophin, nerve growth factor (NGF), neurotrophin-3 (NT-3and brain-derived neurotrophic factor (BDNF)+ NGF and NT-3 promote axonal growth from sensory neurons through activation of their corresponding tyrosine kinase (Trk) receptors, TrkA24, 25 and 26 and TrkC27 and 28, respectively and act naturally' Note' BDNF is known to enhance survival and promote axon growth from retinal ganglion cells and hippocampal neurons via its receptor, TrkB29. NGF encourages axonal extension in vitro 30 and 31 and leads to axonal growth in vivo 32. NGF has even been shown to overcome axon growth inhibition by CSPG in dorsal root ganglion (DRG) neurons 33. Similar to NGF, NT-3 is known to enhance the growth of axons in vivo significantly for both peripheral and central neurons 34, 35, 36 and 37. BDNF has been shown to play an important role in the regulation of synapse structure and function, especially in glutamatergic synapses 38. It has also been demonstrated that BDNF and NT-3 significantly influence axon path-finding, as well as aiding axonal regeneration in rats following spinal cord injury 39 and 40. Moreover, during development these NTs direct the path-finding of maturing axons to their innervating targets by a combination of long-range attractive and repulsive cues41. The functions and importance of these NTs in the NS have recently been reviewed 42.
Evidence that basic fibroblast growth factor (bFGF-2) and glial cell line-derived neurotrophic factor (GDNF) may play a role in facilitating nerve regeneration in the peripheral nervous system (PNS) and CNS. Both growth factors have been shown to influence neurons, Schwann cells, and oligodendrocytes toward axonal growth and remyelination following injury 43 and 44. Ciliary neurotrophic factor (CNTF) has been shown to act primarily on neurons as a survival factor following injury in the PNS 45. so far, CNTF has not demonstrated any functional or regenerative benefits for nerve repair 46, however, it shows helpful signs when calcium ions are added' note; beneficial effects with other neurotrophins;
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