Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

By I. Dragatsis, M. Levine, S. Zeitlin. Nature Genetics, 2000

611 citations recorded by the source corpus.

Subfields: Cellular and Molecular Neuroscience, Molecular Biology

View Paper: https://www.semanticscholar.org/paper/58ae3e8a1548b22dd22d881a10be53491c4e9abb

How researchers see this paper

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In Cellular and Molecular Neuroscience

1. C. Zuccato
2. E. Cattaneo
3. M. Valenza
4. D. Cleveland
5. N. Bonini
6. Volkmar Lessmann

In Molecular Biology

1. D. Cleveland
2. Volkmar Lessmann
3. M. Valenza
4. E. Cattaneo
5. C. Zuccato

Citations in the corpus

• Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. (2003, background): “Since these mice overexpress full-length huntingtin, one possibility is the increased weight is due to the effects of full-length huntingtin on survival of cells (16,26).”
• Molecular mechanisms and potential therapeutical targets in Huntington's disease. (2010, methodology): “In 2000, in parallel to our demonstration of an antiapoptotic role of huntingtin in vitro, work from Scott Zeitlin’s group at the University of Virginia showed that neuronal inactivation of the huntingtin gene in adult mice by means of a Cre/LoxP site-specific recombination system driven by the neur”
• Sustained therapeutic reversal of Huntington’s disease by transient repression of huntingtin synthesis (2012, positive): “5 or postnatal day 5 (Dragatsis et al., 2000) and in cultured neuronal cells (Gauthier et al.”
• Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila. (2003, positive): “In mice, deletion of the huntingtin gene results in early embryonic lethality, whereas later deletion of huntingtin by conditional mutagenesis causes neuronal degeneration (Dragatsis et al., 2000; Duyao et al., 1995;Summary Nasir et al., 1995; Zeitlin et al., 1995).”
• Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules. (2004, background): “These beneficial properties of wild-type htt are necessary in adults, as late inactivation of htt in a conditional mouse modelSummary leads to progressive neurodegeneration (Dragatsis et al., 2000).”