| The Drosophila homologue of the Angelman syndrome ubiquitin ligase regulates the formation of terminal dendritic branches. | |
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MedLine Citation:
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PMID: 18996915 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Angelman syndrome is a severe neurodevelopmental disorder mostly caused by loss-of-function mutations in the maternal allele of UBE3A, a gene that encodes an E3 ubiquitin ligase. Drosophila UBE3A (dUBE3A) is highly homologous to human UBE3A (hUBE3A) at the amino acid sequence level, suggesting their functional conservation. We generated dUBE3A-null mutant fly lines and found that dUBE3A is not essential for viability. However, loss of dUBE3A activity reduced dendritic branching of sensory neurons in the peripheral nervous system and slowed the growth of terminal dendritic fine processes. Several lines of evidence indicated that dUBE3A regulates dendritic morphogenesis in a cell autonomous manner. Moreover, overexpression of dUBE3A also decreased dendritic branching, suggesting that the proper level of dUBE3A is critically important for the normal dendritic patterning. These findings suggest that dendritic pathology may contribute to neurological deficits in patients with Angelman syndrome. |
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Authors:
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Yubing Lu; Fay Wang; Yan Li; Jacob Ferris; Jin-A Lee; Fen-Biao Gao |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2008-11-07 |
Journal Detail:
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Title: Human molecular genetics Volume: 18 ISSN: 1460-2083 ISO Abbreviation: Hum. Mol. Genet. Publication Date: 2009 Feb |
Date Detail:
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Created Date: 2009-01-15 Completed Date: 2009-02-27 Revised Date: 2010-09-22 |
Medline Journal Info:
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Nlm Unique ID: 9208958 Medline TA: Hum Mol Genet Country: England |
Other Details:
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Languages: eng Pagination: 454-62 Citation Subset: IM |
Affiliation:
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Gladstone Institute of Neurological Disease, San Francisco, CA 94158, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Angelman Syndrome
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enzymology,
genetics Animals Dendrites / enzymology*, genetics Drosophila / enzymology*, genetics, growth & development Drosophila Proteins / genetics, metabolism* Humans Morphogenesis Peripheral Nervous System / cytology, enzymology, growth & development Sensory Receptor Cells / cytology, enzymology Ubiquitin-Protein Ligases / genetics, metabolism* |
| Grant Support | |
ID/Acronym/Agency:
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HD044752/HD/NICHD NIH HHS; MH079198/MH/NIMH NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Drosophila Proteins; EC 6.3.2.19/Ube3a protein, Drosophila; EC 6.3.2.19/Ubiquitin-Protein Ligases |
| Comments/Corrections | |
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