| Home > Publications Database > Lack of Pur-alpha alters postnatal brain development and causes megalencephaly. > print |
| 001 | 136414 | ||
| 005 | 20240321220050.0 | ||
| 024 | 7 | _ | |a 10.1093/hmg/ddr476 |2 doi |
| 024 | 7 | _ | |a pmid:22010047 |2 pmid |
| 024 | 7 | _ | |a 0964-6906 |2 ISSN |
| 024 | 7 | _ | |a 1460-2083 |2 ISSN |
| 024 | 7 | _ | |a altmetric:3193076 |2 altmetric |
| 037 | _ | _ | |a DZNE-2020-02736 |
| 041 | _ | _ | |a English |
| 082 | _ | _ | |a 570 |
| 100 | 1 | _ | |a Hokkanen, Suvi |0 P:(DE-2719)9000134 |b 0 |e First author |
| 245 | _ | _ | |a Lack of Pur-alpha alters postnatal brain development and causes megalencephaly. |
| 260 | _ | _ | |a Oxford |c 2012 |b Oxford Univ. Press |
| 264 | _ | 1 | |3 online |2 Crossref |b Oxford University Press (OUP) |c 2011-10-18 |
| 264 | _ | 1 | |3 print |2 Crossref |b Oxford University Press (OUP) |c 2012-02-01 |
| 336 | 7 | _ | |a article |2 DRIVER |
| 336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1589802855_26886 |2 PUB:(DE-HGF) |
| 336 | 7 | _ | |a ARTICLE |2 BibTeX |
| 336 | 7 | _ | |a JOURNAL_ARTICLE |2 ORCID |
| 336 | 7 | _ | |a Journal Article |0 0 |2 EndNote |
| 520 | _ | _ | |a 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. |
| 536 | _ | _ | |a 342 - Disease Mechanisms and Model Systems (POF3-342) |0 G:(DE-HGF)POF3-342 |c POF3-342 |f POF III |x 0 |
| 588 | _ | _ | |a Dataset connected to CrossRef, PubMed, |
| 650 | _ | 7 | |a DNA-Binding Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a Microtubule-Associated Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a Nerve Tissue Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a Neurofilament Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a Pura protein, mouse |2 NLM Chemicals |
| 650 | _ | 2 | |a Animals |2 MeSH |
| 650 | _ | 2 | |a Axons: metabolism |2 MeSH |
| 650 | _ | 2 | |a Brain: growth & development |2 MeSH |
| 650 | _ | 2 | |a Brain: pathology |2 MeSH |
| 650 | _ | 2 | |a Brain Chemistry |2 MeSH |
| 650 | _ | 2 | |a Cell Proliferation |2 MeSH |
| 650 | _ | 2 | |a Cerebellum: cytology |2 MeSH |
| 650 | _ | 2 | |a Cerebellum: growth & development |2 MeSH |
| 650 | _ | 2 | |a Cerebellum: pathology |2 MeSH |
| 650 | _ | 2 | |a Cerebrum: growth & development |2 MeSH |
| 650 | _ | 2 | |a Cerebrum: pathology |2 MeSH |
| 650 | _ | 2 | |a DNA-Binding Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a DNA-Binding Proteins: physiology |2 MeSH |
| 650 | _ | 2 | |a Hippocampus: cytology |2 MeSH |
| 650 | _ | 2 | |a Hippocampus: growth & development |2 MeSH |
| 650 | _ | 2 | |a Hypertrophy |2 MeSH |
| 650 | _ | 2 | |a Mice |2 MeSH |
| 650 | _ | 2 | |a Mice, Knockout |2 MeSH |
| 650 | _ | 2 | |a Microtubule-Associated Proteins: analysis |2 MeSH |
| 650 | _ | 2 | |a Nerve Tissue Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a Nerve Tissue Proteins: physiology |2 MeSH |
| 650 | _ | 2 | |a Neurofilament Proteins: metabolism |2 MeSH |
| 650 | _ | 2 | |a Phosphorylation |2 MeSH |
| 700 | 1 | _ | |a Feldmann, Heidi M |b 1 |
| 700 | 1 | _ | |a Ding, Haiyan |b 2 |
| 700 | 1 | _ | |a Jung, Christian K E |0 P:(DE-2719)9000145 |b 3 |
| 700 | 1 | _ | |a Bojarski, Lukasz |b 4 |
| 700 | 1 | _ | |a Renner-Müller, Ingrid |b 5 |
| 700 | 1 | _ | |a Schüller, Ulrich |0 P:(DE-2719)9000979 |b 6 |u dzne |
| 700 | 1 | _ | |a Kretzschmar, Hans |0 P:(DE-2719)9000168 |b 7 |
| 700 | 1 | _ | |a Wolf, Eckhard |b 8 |
| 700 | 1 | _ | |a Herms, Jochen |0 P:(DE-2719)2810441 |b 9 |e Last author |
| 773 | 1 | 8 | |a 10.1093/hmg/ddr476 |b : Oxford University Press (OUP), 2011-10-18 |n 3 |p 473-484 |3 journal-article |2 Crossref |t Human Molecular Genetics |v 21 |y 2011 |x 0964-6906 |
| 773 | _ | _ | |a 10.1093/hmg/ddr476 |g Vol. 21, no. 3, p. 473 - 484 |0 PERI:(DE-600)1474816-2 |n 3 |q 21:3<473 - 484 |p 473-484 |t Human molecular genetics |v 21 |y 2011 |x 0964-6906 |
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