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@ARTICLE{Zhang:302880,
      author       = {L. Zhang and A. Pouya and J. Kopetzky and S. Bitar and C.
                      Wolf and F. D. Bello and D. Gómez-Zepeda$^*$ and S.
                      Tenzer$^*$ and A. Methner},
      title        = {{I}ncreased {BNIP}3-mediated mitophagy attenuates {GDAP}1
                      loss of function - implications for
                      {C}harcot-{M}arie-{T}ooth disease 4{A}.},
      journal      = {Neurobiology of disease},
      volume       = {213},
      issn         = {0969-9961},
      address      = {[Amsterdam]},
      publisher    = {Elsevier},
      reportid     = {DKFZ-2025-01420},
      pages        = {107019},
      year         = {2025},
      abstract     = {Charcot-Marie-Tooth disease type 4 A ((CMT4A), an autosomal
                      recessive neuropathy, is caused by mutations in
                      ganglioside-induced differentiation-associated protein 1
                      (GDAP1). GDAP1 resides in the outer mitochondrial membrane
                      facing the cytosol and is involved in mitochondrial dynamics
                      and function. Its perturbation affects mitochondrial shape,
                      contact sites, redox homeostasis and cellular metabolism. In
                      response to GDAP1 knockdown in a human neuronal cell line,
                      we found increased mitochondrial turnover, biogenesis and
                      mitophagy. This was associated with more lysosomal proteins
                      in mitochondrial fractions including BCL2/adenovirus E1B 19
                      kDa protein-interacting protein 3 (BNIP3) and its homolog
                      BNIP3-like (BNIP3L) - proteins involved in the recruitment
                      of autophagy machinery via direct interaction. Flies with
                      neural Gdap1 knockdown also exhibited upregulated levels of
                      the sole BNIP3 ortholog. Neural expression of human BNIP3
                      reduced the detrimental effects of Gdap1 knockdown on
                      eclosion and climbing ability in adult flies, while
                      simultaneous knockdown of both genes was detrimental. These
                      findings suggest that increased BNIP3-driven mitophagy may
                      act as a protective mechanism, partially counteracting the
                      cellular dysfunction caused by GDAP1 loss of function, and
                      highlight the potential of targeting mitophagy pathways as a
                      therapeutic strategy for CMT4A.},
      keywords     = {BNIP3 (Other) / Charcot-Marie-tooth (CMT) disease (Other) /
                      Drosophila (Other) / GDAP1 (Other) / Mitophagy (Other)},
      cin          = {D191 / D190},
      ddc          = {570},
      cid          = {I:(DE-He78)D191-20160331 / I:(DE-He78)D190-20160331},
      pnm          = {314 - Immunologie und Krebs (POF4-314)},
      pid          = {G:(DE-HGF)POF4-314},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:40618856},
      doi          = {10.1016/j.nbd.2025.107019},
      url          = {https://inrepo02.dkfz.de/record/302880},
}