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  New kid on the ID block: Neural functions of the Nab2/ZC3H14 class of Cys3His tandem zinc-finger polyadenosine RNA binding proteins

Kelly, S., Pak, C., Garshasbi, M., Kuss, A., Corbett, A. H., & Moberg, K. (2012). New kid on the ID block: Neural functions of the Nab2/ZC3H14 class of Cys3His tandem zinc-finger polyadenosine RNA binding proteins. RNA Biology, 9(5), 555-562. doi:10.4161/rna.20187.

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Item Permalink: http://hdl.handle.net/11858/00-001M-0000-000E-EC74-A Version Permalink: http://hdl.handle.net/11858/00-001M-0000-000E-EC75-8
Genre: Journal Article

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© 2012 Landes Bioscience
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 Creators:
Kelly, S., Author
Pak, C., Author
Garshasbi, M.1, 2, Author              
Kuss, A.3, Author              
Corbett, A. H., Author
Moberg, K., Author
Affiliations:
1Dept. of Human Molecular Genetics (Head: Hans-Hilger Ropers), Max Planck Institute for Molecular Genetics, Max Planck Society, Berlin, Germany, ou_1433549              
2Department of Medical Genetics; Faculty of Medical Sciences; Tarbiat Modares University, Tehran, Iran , ou_persistent22              
3Familial Cognitive Disorders (Luciana Musante), Dept. of Human Molecular Genetics (Head: Hans-Hilger Ropers), Max Planck Institute for Molecular Genetics, Max Planck Society, ou_1479644              

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Free keywords: Drosophila melanogaster, intellectual disability, polyadenylation, post-transcriptional RNA processing, zinc finger polyadenosine RNA binding protein
 Abstract: Polyadenosine RNA binding proteins (Pabs) play critical roles in regulating the polyadenylation, nuclear export, stability, and translation of cellular RNAs. Although most Pabs are ubiquitously expressed and are thought to play general roles in post-transcriptional regulation, mutations in genes encoding these factors have been linked to tissue-specific diseases including muscular dystrophy and now intellectual disability (ID). Our recent work defined this connection to ID, as we showed that mutations in the gene encoding the ubiquitously expressed Cys3His tandem zinc-finger (ZnF) Pab, ZC3H14 (Zinc finger protein, CCCH-type, number 14) are associated with non-syndromic autosomal recessive intellectual disability (NS-ARID). This study provided a first link between defects in Pab function and a brain disorder, suggesting that ZC3H14 plays a required role in regulating RNAs in nervous system cells. Here we highlight key questions raised by our study of ZC3H14 and its ortholog in the fruit fly Drosophila melanogaster, dNab2, and comment on future approaches that could provide insights into the cellular and molecular roles of this class of zinc finger-containing Pabs. We propose a summary model depicting how ZC3H14-type Pabs might play particularly important roles in neuronal RNA metabolism.

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Language(s): eng - English
 Dates: 2012-05-012012-05
 Publication Status: Published in print
 Pages: -
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 Table of Contents: -
 Rev. Type: Peer
 Identifiers: DOI: 10.4161/rna.20187
 Degree: -

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Title: RNA Biology
Source Genre: Journal
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Publ. Info: Georgetown, TX : Landes Bioscience
Pages: - Volume / Issue: 9 (5) Sequence Number: - Start / End Page: 555 - 562 Identifier: ISSN: 1547-6286
CoNE: https://pure.mpg.de/cone/journals/resource/1000000000021460