AAV-mediated cardiac gene transfer of wild-type desmin in mouse models for recessive desminopathies

  1. Ruppert, T.
  2. Heckmann, Markus Benjamin ORCID logo
  3. Rapti, K.
  4. Schultheis, D.
  5. Jungmann, A.
  6. Katus, H. A.
  7. Winter, L.
  8. Frey, N.
  9. Clemen, Christoph S. ORCID logo
  10. Schröder, R.
  11. Müller, Oliver ORCID logo

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1000 Titel
  • AAV-mediated cardiac gene transfer of wild-type desmin in mouse models for recessive desminopathies
1000 Autor/in
  1. Ruppert, T. |
  2. Heckmann, Markus Benjamin |
  3. Rapti, K. |
  4. Schultheis, D. |
  5. Jungmann, A. |
  6. Katus, H. A. |
  7. Winter, L. |
  8. Frey, N. |
  9. Clemen, Christoph S. |
  10. Schröder, R. |
  11. Müller, Oliver |
1000 Erscheinungsjahr 2020
1000 Publikationstyp
  1. Artikel |
1000 Online veröffentlicht
  • 2020-04-22
1000 Erschienen in
1000 Quellenangabe
  • 27(10-11):516-524
1000 Copyrightjahr
  • 2020
1000 Lizenz
1000 Verlagsversion
  • https://doi.org/10.1038/s41434-020-0147-7 |
  • https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7674151/ |
1000 Publikationsstatus
1000 Sprache der Publikation
1000 Abstract/Summary
  • Mutations in the human desmin gene cause autosomal-dominant and recessive cardiomyopathies and myopathies with marked phenotypic variability. Here, we investigated the effects of adeno-associated virus (AAV)-mediated cardiac wild-type desmin expression in homozygous desmin knockout (DKO) and homozygous R349P desmin knockin (DKI) mice. These mice serve as disease models for two subforms of autosomal-recessive desminopathies, the former for the one with a complete lack of desmin protein and the latter for the one with solely mutant desmin protein expression in conjunction with protein aggregation pathology in striated muscle. Two-month-old mice were injected with either a single dose of 5 × 10
1000 Sacherschließung
lokal Genetic Therapy [MeSH]
lokal Mice [MeSH]
lokal Article
lokal Gene therapy
lokal Dependovirus/genetics [MeSH]
lokal Desmin/genetics [MeSH]
lokal Humans [MeSH]
lokal Cardiovascular diseases
lokal Animals [MeSH]
lokal Cardiomyopathies/therapy [MeSH]
lokal Disease Models, Animal [MeSH]
lokal Cardiomyopathies/genetics [MeSH]
1000 Liste der Beteiligten
  1. https://frl.publisso.de/adhoc/uri/UnVwcGVydCwgVC4=|https://orcid.org/0000-0002-3085-1282|https://frl.publisso.de/adhoc/uri/UmFwdGksIEsu|https://frl.publisso.de/adhoc/uri/U2NodWx0aGVpcywgRC4=|https://frl.publisso.de/adhoc/uri/SnVuZ21hbm4sIEEu|https://frl.publisso.de/adhoc/uri/S2F0dXMsIEguIEEu|https://frl.publisso.de/adhoc/uri/V2ludGVyLCBMLg==|https://frl.publisso.de/adhoc/uri/RnJleSwgTi4=|https://orcid.org/0000-0002-1291-4219|https://frl.publisso.de/adhoc/uri/U2NocsO2ZGVyLCBSLg==|https://orcid.org/0000-0001-8223-2638
1000 Hinweis
  • DeepGreen-ID: 9b5a9d3e4e8e4faf8b0b261b7c31f45a ; metadata provieded by: DeepGreen (https://www.oa-deepgreen.de/api/v1/), LIVIVO search scope life sciences (http://z3950.zbmed.de:6210/livivo), Crossref Unified Resource API (https://api.crossref.org/swagger-ui/index.html), to.science.api (https://frl.publisso.de/), ZDB JSON-API (beta) (https://zeitschriftendatenbank.de/api/), lobid - Dateninfrastruktur für Bibliotheken (https://lobid.org/resources/search)
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1000 Erstellt am 2023-11-18T17:29:24.112+0100
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1000 Zuletzt bearbeitet 2024-04-04T10:55:33.898+0200
1000 Objekt bearb. Thu Apr 04 10:55:33 CEST 2024
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  1. oai:frl.publisso.de:frl:6472012 |
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