RGD Reference Report - Fragile X mental retardation protein: nucleocytoplasmic shuttling and association with somatodendritic ribosomes. - Rat Genome Database

RGD Reference Report - Fragile X mental retardation protein: nucleocytoplasmic shuttling and association with somatodendritic ribosomes. - Rat Genome Database

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General

Fragile X mental retardation protein: nucleocytoplasmic shuttling and association with somatodendritic ribosomes.
Authors:	Feng, Y Gutekunst, CA Eberhart, DE Yi, H Warren, ST Hersch, SM
Citation:	Feng Y, etal., J Neurosci. 1997 Mar 1;17(5):1539-47.
RGD ID:	11041080
Pubmed:	PMID:9030614 (View Abstract at PubMed)
PMCID:	PMC6573369 (View Article at PubMed Central)
Fragile X syndrome, a leading cause of inherited mental retardation, is attributable to the unstable expansion of a CGG-repeat within the FMR1 gene that results in the absence of the encoded protein. The fragile X mental retardation protein (FMRP) is a ribosome-associated RNA-binding protein of uncertain function that contains nuclear localization and export signals. We show here detailed cellular localization studies using both biochemical and immunocytochemical approaches. FMRP was highly expressed in neurons but not glia throughout the rat brain, as detected by light microscopy. Although certain structures, such as hippocampus, revealed a strong signal, the regional variation in staining intensity appeared to be related to neuron size and density. In human cell lines and mouse brain, FMRP co-fractionated primarily with polysomes and rough endoplasmic reticulum. Ultrastructural studies in rat brain revealed high levels of FMRP immunoreactivity in neuronal perikarya, where it is concentrated in regions rich in ribosomes, particularly near or between rough endoplasmic reticulum cisternae. Immunogold studies also provided evidence of nucleocytoplasmic shuttling of FMRP, which was localized in neuronal nucleoplasm and within nuclear pores. Moreover, labeling was observed in large- and small-caliber dendrites, in dendritic branch points, at the origins of spine necks, and in spine heads, all known locations of neuronal polysomes. Dendritic localization, which was confirmed by co-fractionation of FMRP with synaptosomal ribosomes, suggests a possible role of FMRP in the translation of proteins involved in dendritic structure or function and relevant for the mental retardation occurring in fragile X syndrome.

Gene Ontology Annotations Click to see Annotation Detail View

Cellular Component

Only show annotations with direct experimental evidence (0 objects hidden)

Term	Qualifier	Evidence	With	Reference	Notes	Source	Original Reference(s)
axon terminus	located_in	IDA		11041080	PMID:9030614	UniProt
dendrite	located_in	IDA		11041080	PMID:9030614	UniProt
dendritic spine	located_in	IDA		11041080	PMID:9030614	UniProt
nucleoplasm		IDA		11041080		RGD
nucleus	located_in	IDA		11041080	PMID:9030614	UniProt
perikaryon	located_in	IDA		11041080	PMID:9030614	UniProt
spine apparatus		IDA		11041080		RGD

Objects Annotated

Genes (Rattus norvegicus)

Fmr1 (fragile X messenger ribonucleoprotein 1)

Additional Information