A Novel RNA-binding Nuclear Protein That Interacts With the Fragile X Mental Retardation (FMR1) Protein

Silenced expression of the FMR1 gene is responsible for the fragile X syndrome. The FMR1 gene codes for an RNA binding protein (FMRP), which can shuttle between the nucleus and the cytoplasm and is found associated to polysomes in the cytoplasm. By two-hybrid assay in yeast, we identified a novel pr...

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Bibliographic Details
Published in:Human molecular genetics 1999-12, Vol.8 (13), p.2557-2566
Main Authors: Bardoni, Barbara, Schenck, Annette, Louis Mandel, Jean
Format: Article
Language:English
Subjects:
Amino Acid Sequence
Animals
Biochemistry, Molecular Biology
Biological and medical sciences
Brain - metabolism
Cell Nucleus - metabolism
Chromosome fragility (bloom syndrome, ataxia telangiectasia, fanconi anemia, x-linked mental retardation...)
COS Cells
Fluorescent Antibody Technique
FMR1 gene
FMR1 protein
Fragile X Mental Retardation Protein
fragile X syndrome
Fragile X Syndrome - genetics
FXR1 gene
FXR2 gene
Gene Expression
HeLa Cells
Humans
In Situ Hybridization
Intellectual Disability - genetics
Life Sciences
Medical genetics
Medical sciences
Mice
Microscopy, Confocal
Molecular Sequence Data
Nerve Tissue Proteins - genetics
Nerve Tissue Proteins - metabolism
Nuclear Proteins - genetics
Nuclear Proteins - metabolism
NUFIP gene
NUFIP protein
Recombinant Proteins - metabolism
RNA-Binding Proteins - genetics
RNA-Binding Proteins - metabolism
Two-Hybrid System Techniques
Yeasts - genetics
Zinc Fingers
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Summary:Silenced expression of the FMR1 gene is responsible for the fragile X syndrome. The FMR1 gene codes for an RNA binding protein (FMRP), which can shuttle between the nucleus and the cytoplasm and is found associated to polysomes in the cytoplasm. By two-hybrid assay in yeast, we identified a novel protein interacting with FMRP: nuclear FMRP interacting protein (NUFIP). NUFIP mRNA expression is strikingly similar to that of the FMR1 gene in neurones of cortex, hippocampus and cerebellum. At the subcellular level, NUFIP colocalizes with nuclear isoforms of FMRP in a dot-like pattern. NUFIP presents a C2H2 zinc finger motif and a nuclear localization signal, but has no homology to known proteins and shows RNA binding activity in vitro. NUFIP does not interact with the FMRP homologues encoded by the FXR1 and FXR2genes. Thus, these results indicate a specific nuclear role for FMRP.
ISSN:0964-6906
1460-2083
DOI:10.1093/hmg/8.13.2557