| Chaperoning ribonucleoprotein biogenesis in health and disease. | |
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MedLine Citation:
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PMID: 17401408 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The survival motor neuron (SMN) protein is part of a macromolecular complex that functions in the biogenesis of small nuclear ribonucleoproteins (snRNPs)--the essential components of the pre-messenger RNA splicing machinery--as well as probably other RNPs. Reduced levels of SMN expression cause the inherited motor neuron disease spinal muscular atrophy (SMA). Knowledge of the composition, interactions and functions of the SMN complex has advanced greatly in recent years. The emerging picture is that the SMN complex acts as a macromolecular chaperone of RNPs to increase the efficiency and fidelity of RNA-protein interactions in vivo, and to provide an opportunity for these interactions to be regulated. In addition, it seems that RNA metabolism deficiencies underlie SMA. Here, a dual dysfunction hypothesis is presented in which two mechanistically and temporally distinct defects--that are dependent on the extent of SMN reduction in SMA--affect the homeostasis of specific messenger RNAs encoding proteins essential for motor neuron development and function. |
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Authors:
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Livio Pellizzoni |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: EMBO reports Volume: 8 ISSN: 1469-221X ISO Abbreviation: EMBO Rep. Publication Date: 2007 Apr |
Date Detail:
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Created Date: 2007-04-02 Completed Date: 2007-05-30 Revised Date: 2011-11-24 |
Medline Journal Info:
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Nlm Unique ID: 100963049 Medline TA: EMBO Rep Country: England |
Other Details:
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Languages: eng Pagination: 340-5 Citation Subset: IM |
Affiliation:
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Dulbecco Telethon Institute, Institute of Cell Biology (CNR), Via E. Ramarini 32, 00016 Monterotondo Scalo, Rome, Italy. livio.pellizzoni@ibc.cnr.it |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Cell Survival Cyclic AMP Response Element-Binding Protein / genetics, metabolism* Health Humans Molecular Chaperones / metabolism* Motor Neurons / metabolism, physiology* Muscular Atrophy, Spinal / etiology*, genetics Nerve Tissue Proteins / genetics, metabolism* Nervous System / cytology, growth & development*, metabolism RNA, Messenger / metabolism RNA-Binding Proteins / genetics, metabolism* Ribonucleoproteins, Small Nuclear / metabolism* SMN Complex Proteins |
| Grant Support | |
ID/Acronym/Agency:
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TCP02011//Telethon |
| Chemical | |
Reg. No./Substance:
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0/Cyclic AMP Response Element-Binding Protein; 0/Molecular Chaperones; 0/Nerve Tissue Proteins; 0/RNA, Messenger; 0/RNA-Binding Proteins; 0/Ribonucleoproteins, Small Nuclear; 0/SMN Complex Proteins |
| Comments/Corrections | |
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