% IMPORTANT: The following is UTF-8 encoded.  This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.

@ARTICLE{Hagiwara:142899,
      author       = {D. Hagiwara$^*$ and V. Grinevich$^*$ and H. Arima},
      title        = {{A} novel mechanism of autophagy-associated cell death of
                      vasopressin neurons in familial neurohypophysial diabetes
                      insipidus.},
      journal      = {Cell $\&$ tissue research},
      volume       = {375},
      number       = {1},
      issn         = {1432-0878},
      address      = {Heidelberg},
      publisher    = {Springer},
      reportid     = {DKFZ-2019-00529},
      pages        = {259 - 266},
      year         = {2019},
      abstract     = {Familial neurohypophysial diabetes insipidus (FNDI),
                      characterized by delayed-onset progressive polyuria and loss
                      of arginine vasopressin (AVP) neuron, is an autosomal
                      dominant disorder caused by AVP gene mutations. We
                      previously generated a knock-in mouse model for FNDI, which
                      recapitulated the phenotype of human FNDI. To address the
                      mechanisms underlying AVP neuron loss, we subjected FNDI
                      mice to intermittent water deprivation, which accelerated
                      the phenotype and induced AVP neuron loss within a relative
                      short period. Electron microscopic analyses revealed that
                      aggregates were confined to a sub-compartment of the
                      endoplasmic reticulum (ER), ER-associated compartment
                      (ERAC), in AVP neurons of FNDI mice under normal conditions.
                      In contrast, aggregates scattered throughout the dilated ER
                      lumen, and phagophores, autophagosome precursors, emerged
                      and surrounded the ER containing scattered aggregates in
                      FNDI mice subjected to water deprivation for 4 weeks,
                      suggesting that failure of ERAC formation leads to autophagy
                      induction for degradation of aggregates. Furthermore, the
                      cytoplasm was entirely occupied with large vacuoles in AVP
                      neurons of FNDI mice subjected to water deprivation for
                      12 weeks, at which stage $30-40\%$ of AVP neurons were
                      lost. Our data demonstrated that although autophagy should
                      primarily be a protective mechanism, continuous autophagy
                      leads to gradual loss of organelles including ER, resulting
                      in autophagy-associated cell death of AVP neurons in FNDI
                      mice.},
      subtyp        = {Review Article},
      cin          = {V078},
      ddc          = {610},
      cid          = {I:(DE-He78)V078-20160331},
      pnm          = {319H - Addenda (POF3-319H)},
      pid          = {G:(DE-HGF)POF3-319H},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:29961215},
      doi          = {10.1007/s00441-018-2872-4},
      url          = {https://inrepo02.dkfz.de/record/142899},
}