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@ARTICLE{Yamoah:1006648,
      author       = {Yamoah, Alfred and Tripathi, Priyanka and Guo, Haihong and
                      Scheve, Leonie and Walter, Peter and Johnen, Sandra and
                      Müller, Frank and Weis, Joachim and Goswami, Anand},
      title        = {{E}arly {A}lterations of {RNA} {B}inding {P}rotein ({RBP})
                      {H}omeostasis and {ER} {S}tress-{M}ediated {A}utophagy
                      {C}ontributes to {P}rogressive {R}etinal {D}egeneration in
                      the rd10 {M}ouse {M}odel of {R}etinitis {P}igmentosa ({RP})},
      journal      = {Cells},
      volume       = {12},
      number       = {7},
      issn         = {2073-4409},
      address      = {Basel},
      publisher    = {MDPI},
      reportid     = {FZJ-2023-01780},
      pages        = {1094},
      year         = {2023},
      note         = {This research was supported by the DFG grants, WE1406/14-3
                      to J.W., WE 1406/16-1 to J.W.and A.G., MU-3036/3-3 to
                      F.M.,WA-1472/6-3 to P.W., and JO-1263/1-3 to S.J., by the
                      InterdisciplinaryCentre for Clinical Research (IZKF Aachen,
                      N7-4, to A.G. and J.W.) and by the EU Joint
                      ProgramNeurodegenerative Disease Research (JPND, Fly-SMALS,
                      to J.W.).},
      abstract     = {The retinal degeneration 10 (rd10) mouse model is widely
                      used to study retinitis pigmentosa(RP) pathomechanisms. It
                      offers a rather unique opportunity to study trans-neuronal
                      degenerationbecause the cell populations in question are
                      separated anatomically and the mutated Pde6b gene
                      isselectively expressed in rod photoreceptors. We
                      hypothesized that RNA binding protein (RBP) aggregationand
                      abnormal autophagy might serve as early pathogenic events,
                      damaging non-photoreceptorretinal cell types that are not
                      primarily targeted by the Pde6b gene defect. We used a
                      combination ofimmunohistochemistry (DAB,
                      immunofluorescence), electron microscopy (EM), subcellular
                      fractionation,andWestern blot analysis on the retinal
                      preparations obtained from both rd10 and wild-typemice. We
                      found early, robust increases in levels of the protective
                      endoplasmic reticulum (ER) calcium(Ca2+) buffering chaperone
                      Sigma receptor 1 (SigR1) together with other ER-Ca2+
                      buffering proteinsin both photoreceptors and
                      non-photoreceptor neuronal cells before any noticeable
                      photoreceptordegeneration. In line with this, we found
                      markedly altered expression of the autophagy proteinsp62 and
                      LC3, together with abnormal ER widening and large autophagic
                      vacuoles as detected byEM. Interestingly, these changes were
                      accompanied by early, prominent cytoplasmic and
                      nuclearaggregation of the key RBPs including pTDP-43 and FET
                      family RBPs and stress granule formation.We conclude that
                      progressive neurodegeneration in the rd10 mouse retina is
                      associated with earlydisturbances of proteostasis and
                      autophagy, along with abnormal cytoplasmic RBP aggregation.},
      cin          = {IBI-1},
      ddc          = {570},
      cid          = {I:(DE-Juel1)IBI-1-20200312},
      pnm          = {5241 - Molecular Information Processing in Cellular Systems
                      (POF4-524)},
      pid          = {G:(DE-HGF)POF4-5241},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {37048167},
      UT           = {WOS:000969574200001},
      doi          = {10.3390/cells12071094},
      url          = {https://juser.fz-juelich.de/record/1006648},
}