Abstract
Nova onconeural antigens are neuron-specific RNA-binding proteins implicated in paraneoplastic opsoclonus-myoclonus-ataxia (POMA) syndrome. Nova harbors three K-homology (KH) motifs implicated in alternate splicing regulation of genes involved in inhibitory synaptic transmission. We report the crystal structure of the first two KH domains (KH1/2) of Nova-1 bound to an in vitro selected RNA hairpin, containing a UCAG-UCAC high-affinity binding site. Sequence-specific intermolecular contacts in the complex involve KH1 and the second UCAC repeat, with the RNA scaffold buttressed by interactions between repeats. Whereas the canonical RNA-binding surface of KH2 in the above complex engages in protein-protein interactions in the crystalline state, the individual KH2 domain can sequence-specifically target the UCAC RNA element in solution. The observed antiparallel alignment of KH1 and KH2 domains in the crystal structure of the complex generates a scaffold that could facilitate target pre-mRNA looping on Nova binding, thereby potentially explaining Nova's functional role in splicing regulation.
Copyright © 2011 Elsevier Ltd. All rights reserved.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Alternative Splicing
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Amino Acid Sequence
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Antigens, Neoplasm* / chemistry
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Antigens, Neoplasm* / genetics
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Antigens, Neoplasm* / metabolism
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Base Sequence
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Binding Sites
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Crystallization
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Crystallography, X-Ray
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Electrophoretic Mobility Shift Assay
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Humans
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Magnetic Resonance Spectroscopy
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Models, Molecular
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Molecular Sequence Data
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Nerve Tissue Proteins* / chemistry
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Nerve Tissue Proteins* / genetics
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Nerve Tissue Proteins* / metabolism
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Neuro-Oncological Ventral Antigen
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Neurons / cytology
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Neurons / metabolism*
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Opsoclonus-Myoclonus Syndrome / metabolism*
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Opsoclonus-Myoclonus Syndrome / physiopathology
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Protein Binding
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Protein Structure, Tertiary
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RNA Precursors / chemistry
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RNA Precursors / metabolism*
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RNA, Small Interfering / chemistry
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RNA, Small Interfering / metabolism*
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RNA-Binding Proteins* / chemistry
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RNA-Binding Proteins* / genetics
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RNA-Binding Proteins* / metabolism
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Sequence Homology, Amino Acid
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Solutions / chemistry
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Solutions / metabolism
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Synaptic Transmission / physiology*
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Syndrome
Substances
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Antigens, Neoplasm
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Nerve Tissue Proteins
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Neuro-Oncological Ventral Antigen
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RNA Precursors
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RNA, Small Interfering
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RNA-Binding Proteins
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Solutions