4.6 Review

ER Dynamics and Derangement in Neurological Diseases

Journal

FRONTIERS IN NEUROSCIENCE
Volume 12, Issue -, Pages -

Publisher

FRONTIERS MEDIA SA
DOI: 10.3389/fnins.2018.00091

Keywords

endoplasmic reticulum; ER architecture; ER dynamics; neurological disease; neurodegeneration; NF-Y

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Funding

  1. Ministry of Education, Culture, Sports, Science and Technology (MEXT) [15H01567]
  2. MEXT/The Japan Society for the Promotion of Science (JSPS) [17H01564, 16H01345, 17KT0131, 15K06762]
  3. Takeda Science Foundation
  4. Center for Baby Science in Doshisha University (Joint Usage/Research Center accredited by MEXT)
  5. Japan Agency for Medical Research and Development, AMED
  6. Grants-in-Aid for Scientific Research [15K06762, 15H01567, 16H01345, 17H01564, 17KT0131] Funding Source: KAKEN

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The endoplasmic reticulum (ER) is a morphologically dynamic organelle containing different membrane subdomains with distinct cellular functions. Numerous observations have revealed that ER stress response induced by disturbed ER homeostasis is linked to various neurological/neurodegenerative disorders. In contrast, recent findings unveil that ER structural derangements are linked to the progression of several neurological diseases. The derangements involve two distinct, and likely opposing pathways. One is dysfunction of ER dynamics machinery, leading to disruption of ER network organization. Another one is facilitation of pre-existing machinery, leading to generation of markedly-ordered de novo membranous structure. Restoring the ER network can be the effective way toward the cure of ER-deranged neurological disorders.

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