Pathogenic Mechanisms in Neuroimmunodegeneration

  • William S. Lynn
  • Paul K. Y. Wong


The number of signals now known to modulate cell survival and death of neuronal and immune (peripheral and central) cells is increasing rapidly. The timing and concentration of these signals are especially crucial during postnatal development when rapid adaptation to ever-changing environmental signals is required. During this period, cell growth must be carefully balanced with cell death, and cell differentiation must be commensurate with the rapidly changing environmental signals, especially those signals produced by nutrients or by their interacting neighboring cells and matrix. The number and activity of each of the cellular organelles must also be carefully adjusted to meet the specific and rapidly changing needs of the cells. Interactions between the cells and their neighboring cells and matrix must also be exquisitely programmed. During this period of interactive, postnatal development, errors and imbalances are frequent. Under situations of either inborn errors (e.g., genetic defects) or of excessive environmental stresses (e.g., viral infections), unrepaired genetic mistakes and indigestible oxidized peptides accumulate. Cell death, either apoptotic or oxidative, especially in the immune and nervous systems, becomes imminent. In the neuroimmunodegeneration (NID) syndromes outlined in chapter 2, cell death is cell induced (apoptotic), and therefore may not alert the peripheral migratory immune system but usually does alert the CNS immune/support system. The initial response of the CNS immune/support system is to defend and protect the apoptotic neurons. However, the defensive maneuvers of the CNS immune/support cells may become excessive and usurp their neuronal support functions. The loss of neuronal support as a result of impaired function of the CNS immune/support cells may account for much of the neuronal death seen in these NID syndromes.


Nitric Oxide Endoplasmic Reticulum Cell Death Pathway Ataxia Telangiectasia NADH Oxidase 
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© Springer-Verlag Berlin Heidelberg 1998

Authors and Affiliations

  • William S. Lynn
  • Paul K. Y. Wong

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