GONUTS has been updated to MW1.31 Most things seem to be working but be sure to report problems.

Have any questions? Please email us at ecoliwiki@gmail.com

PMID:18093976

From GONUTS
Jump to: navigation, search
Citation

Piazzon, N, Rage, F, Schlotter, F, Moine, H, Branlant, C and Massenet, S (2008) In vitro and in cellulo evidences for association of the survival of motor neuron complex with the fragile X mental retardation protein. J. Biol. Chem. 283:5598-610

Abstract

Spinal muscular atrophy (SMA) is caused by reduced levels of the survival of motor neuron (SMN) protein. Although the SMN complex is essential for assembly of spliceosomal U small nuclear RNPs, it is still not understood why reduced levels of the SMN protein specifically cause motor neuron degeneration. SMN was recently proposed to have specific functions in mRNA transport and translation regulation in neuronal processes. The defective protein in Fragile X mental retardation syndrome (FMRP) also plays a role in transport of mRNPs and in their translation. Therefore, we examined possible relationships of SMN with FMRP. We observed granules containing both transiently expressed red fluorescent protein(RFP)-tagged SMN and green fluorescent protein(GFP)-tagged FMRP in cell bodies and processes of rat primary neurons of hypothalamus in culture. By immunoprecipitation experiments, we detected an association of FMRP with the SMN complex in human neuroblastoma SH-SY5Y cells and in murine motor neuron MN-1 cells. Then, by in vitro experiments, we demonstrated that the SMN protein is essential for this association. We showed that the COOH-terminal region of FMRP, as well as the conserved YG box and the region encoded by exon 7 of SMN, are required for the interaction. Our findings suggest a link between the SMN complex and FMRP in neuronal cells.

Links

PubMed Online version:10.1074/jbc.M707304200

Keywords

Animals; Cell Line, Tumor; Cyclic AMP Response Element-Binding Protein/genetics; Cyclic AMP Response Element-Binding Protein/metabolism; Exons/physiology; Fragile X Mental Retardation Protein/genetics; Fragile X Mental Retardation Protein/metabolism; Fragile X Syndrome/genetics; Fragile X Syndrome/metabolism; Fragile X Syndrome/pathology; Humans; Hypothalamus/metabolism; Hypothalamus/pathology; Mice; Motor Neurons/metabolism; Motor Neurons/pathology; Muscular Atrophy, Spinal/genetics; Muscular Atrophy, Spinal/metabolism; Muscular Atrophy, Spinal/pathology; Nerve Tissue Proteins/genetics; Nerve Tissue Proteins/metabolism; Protein Structure, Tertiary/physiology; RNA-Binding Proteins/genetics; RNA-Binding Proteins/metabolism; Recombinant Fusion Proteins/genetics; Recombinant Fusion Proteins/metabolism; SMN Complex Proteins; Survival of Motor Neuron 1 Protein

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

HUMAN:FMR1

enables

GO:0005515: protein binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:Q16637

F

Seeded From UniProt

complete

HUMAN:FMR1

located_in

GO:0043204: perikaryon

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:FMR1

located_in

GO:0036464: cytoplasmic ribonucleoprotein granule

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:FMR1

located_in

GO:0043005: neuron projection

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:SMN

enables

GO:0005515: protein binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:Q06787

F

Seeded From UniProt

complete

HUMAN:SMN

located_in

GO:0043204: perikaryon

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:SMN

located_in

GO:0036464: cytoplasmic ribonucleoprotein granule

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:SMN

located_in

GO:0043005: neuron projection

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:SMN

part_of

GO:0032797: SMN complex

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:FMR1

part_of

GO:0043005: neuron projection

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:FMR1

part_of

GO:0043204: perikaryon

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:FMR1

colocalizes_with

GO:0032797: SMN complex

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:FMR1

part_of

GO:0036464: cytoplasmic ribonucleoprotein granule

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

MOUSE:FMR1

colocalizes_with

GO:0032797: SMN complex

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

Notes

See also

References

See Help:References for how to manage references in GONUTS.