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@ARTICLE{Hokkanen:136414,
      author       = {Hokkanen, Suvi and Feldmann, Heidi M and Ding, Haiyan and
                      Jung, Christian K E and Bojarski, Lukasz and Renner-Müller,
                      Ingrid and Schüller, Ulrich and Kretzschmar, Hans and Wolf,
                      Eckhard and Herms, Jochen},
      title        = {{L}ack of {P}ur-alpha alters postnatal brain development
                      and causes megalencephaly.},
      journal      = {Human molecular genetics},
      volume       = {21},
      number       = {3},
      issn         = {0964-6906},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {DZNE-2020-02736},
      pages        = {473-484},
      year         = {2012},
      abstract     = {Pur-alpha (Purα) plays an important role in a variety of
                      cellular processes including transcriptional regulation,
                      cell proliferation and oncogenic transformation. To better
                      understand the role of Purα in the developing and mature
                      brain, we generated Purα-deficient mice, which we were able
                      to raise to the age of six months. Purα(-/-) mice were born
                      with no obvious pathological condition. We obtained
                      convincing evidence that lack of Purα prolongs the
                      postnatal proliferation of neuronal precursor cells both in
                      the hippocampus and in the cerebellum, however, without
                      affecting the overall number of postmitotic neurons.
                      Independent of these findings, we observed alterations in
                      the expression and distribution of the dendritic protein
                      MAP2, the translation of which has been proposed previously
                      to be Purα-dependent. At the age of 2 weeks, Purα(-/-)
                      mice generated a continuous tremor which persisted
                      throughout lifetime. Finally, adult Purα(-/-) mice
                      displayed a megalencephaly and histopathological findings
                      including axonal swellings and hyperphosphorylation of
                      neurofilaments. Our studies underline the importance of
                      Purα in the proliferation of neuronal precursor cells
                      during postnatal brain development and suggest a role for
                      Purα in the regulation of the expression and cellular
                      distribution of dendritic and axonal proteins. Since recent
                      studies implicate a link between Purα and the fragile X
                      tremor/ataxia syndrome, our Purα(-/-) mouse model will
                      provide new opportunities for understanding the mechanisms
                      of neurodegeneration.},
      keywords     = {Animals / Axons: metabolism / Brain: growth $\&$
                      development / Brain: pathology / Brain Chemistry / Cell
                      Proliferation / Cerebellum: cytology / Cerebellum: growth
                      $\&$ development / Cerebellum: pathology / Cerebrum: growth
                      $\&$ development / Cerebrum: pathology / DNA-Binding
                      Proteins: genetics / DNA-Binding Proteins: physiology /
                      Hippocampus: cytology / Hippocampus: growth $\&$ development
                      / Hypertrophy / Mice / Mice, Knockout /
                      Microtubule-Associated Proteins: analysis / Nerve Tissue
                      Proteins: genetics / Nerve Tissue Proteins: physiology /
                      Neurofilament Proteins: metabolism / Phosphorylation /
                      DNA-Binding Proteins (NLM Chemicals) /
                      Microtubule-Associated Proteins (NLM Chemicals) / Nerve
                      Tissue Proteins (NLM Chemicals) / Neurofilament Proteins
                      (NLM Chemicals) / Pura protein, mouse (NLM Chemicals)},
      cin          = {München Pre 2020 / AG Herms / Ext LMU ZNP},
      ddc          = {570},
      cid          = {I:(DE-2719)6000016 / I:(DE-2719)1110001 /
                      I:(DE-2719)5000051},
      pnm          = {342 - Disease Mechanisms and Model Systems (POF3-342)},
      pid          = {G:(DE-HGF)POF3-342},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:22010047},
      doi          = {10.1093/hmg/ddr476},
      url          = {https://pub.dzne.de/record/136414},
}