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@ARTICLE{Pa:1031535,
      author       = {Paß, Thomas and Ricke, Konrad M and Hofmann, Pierre and
                      Chowdhury, Roy S and Nie, Yu and Chinnery, Patrick and
                      Endepols, Heike and Neumaier, Bernd and Carvalho, André and
                      Rigoux, Lionel and Steculorum, Sophie M and Prudent, Julien
                      and Riemer, Trine and Aswendt, Markus and Liss, Birgit and
                      Brachvogel, Bent and Wiesner, Rudolf J},
      title        = {{P}reserved striatal innervation maintains motor function
                      despite severe loss of nigral dopaminergic neurons},
      journal      = {Brain},
      volume       = {147},
      number       = {9},
      issn         = {0006-8950},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {FZJ-2024-05730},
      pages        = {3189 - 3203},
      year         = {2024},
      note         = {T.P and R.J.W received funds from the Center of Molecular
                      Medicine Cologne (CMMC, C17); K.M.R. received funding from
                      Parkinson Canada Basic Research Fellowship
                      (BRF-2021-0000000048); A.C. received support by the Cologne
                      Graduate School of Ageing Research; R.S.C. and J.P. received
                      funds from the Medical Research Council UK
                      $(MC_UU_00028/5).$ Y.N. and P.C. were supported by a
                      Wellcome Collaborative Award (224486/Z/21/Z), the Medical
                      Research Council Mitochondrial Biology Unit
                      $(MC_UU_00028/7),$ the Medical Research Council (MRC)
                      International Centre for Genomic Medicine in Neuromuscular
                      Disease (MR/S005021/1), the Leverhulme Trust (RPG-2018-408),
                      an MRC research grant (MR/S035699/1), an Alzheimer’s
                      Society Project Grant (AS-PG-18b-022), and the NIHR
                      Cambridge Biomedical Research Centre (BRC-1215-20014); M.A.
                      acknowledges financial support by the Friebe Foundation
                      (T0498/28960/16) and the Deutsche Forschungsgemeinschaft
                      (DFG, German Research Foundation)—Project-ID
                      431549029—SFB 1451.},
      abstract     = {Degeneration of dopaminergic neurons in the substantia
                      nigra and their striatal axon terminals causes cardinal
                      motorsymptoms of Parkinson’s disease. In idiopathic cases,
                      high levels of mitochondrial DNA alterations, leading
                      tomitochondrial dysfunction, are a central feature of these
                      vulnerable neurons.Here we present a mouse model expressing
                      the K320E variant of the mitochondrial helicase Twinkle in
                      dopaminergicneurons, leading to accelerated mitochondrial
                      DNA mutations. These K320E-TwinkleDaN mice showed normal
                      motorfunction at 20 months of age, although $∼70\%$ of
                      nigral dopaminergic neurons had perished. Remaining neurons
                      stillpreserved $∼75\%$ of axon terminals in the dorsal
                      striatum and enabled normal dopamine release. Transcriptome
                      analysisand viral tracing confirmed compensatory axonal
                      sprouting of the surviving neurons.We conclude that a small
                      population of substantia nigra dopaminergic neurons is able
                      to adapt to the accumulation ofmitochondrial DNA mutations
                      and maintain motor control.},
      cin          = {INM-5},
      ddc          = {610},
      cid          = {I:(DE-Juel1)INM-5-20090406},
      pnm          = {5253 - Neuroimaging (POF4-525)},
      pid          = {G:(DE-HGF)POF4-5253},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {38574200},
      UT           = {WOS:001296119400001},
      doi          = {10.1093/brain/awae089},
      url          = {https://juser.fz-juelich.de/record/1031535},
}