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PMID:19464282

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Citation

Lee, L, Davies, SE and Liu, JL (2009) The spinal muscular atrophy protein SMN affects Drosophila germline nuclear organization through the U body-P body pathway. Dev. Biol. 332:142-55

Abstract

Survival motor neuron protein (SMN) is the determining factor for the human neurodegenerative disease spinal muscular atrophy (SMA). SMN is critical for small nuclear ribonucleoprotein (snRNP) assembly. Using Drosophila oogenesis as a model system, we show that mutations in smn cause abnormal nuclear organization in nurse cells and oocytes. Germline and mitotic clonal analysis reveals that both nurse cells and oocytes require SMN to maintain normal organization of nuclear compartments including chromosomes, nucleoli, Cajal bodies and histone locus bodies. We previously found that SMN-containing U bodies invariably associate with P bodies (Liu, J. L., and Gall, J. G. (2007). U bodies are cytoplasmic structures that contain uridine-rich small nuclear ribonucleoproteins and associate with P bodies. Proc. Natl. Acad. Sci. U. S. A. 104, 11655-11659.). Multiple lines of evidence implicate SMN in the regulation of germline nuclear organization through the connection of U bodies and P bodies. Firstly, smn germline clones phenocopy mutations for two P body components, Cup and Ovarian tumour (Otu). Secondly, P body mutations disrupt SMN distribution and the organization of U bodies. Finally, mutations in smn disrupt the function and organization of U bodies and P bodies. Taken together, our results suggest that SMN is required for the functional integrity of the U body-P body pathway, which in turn is important for maintaining proper nuclear architecture.

Links

PubMed Online version:10.1016/j.ydbio.2009.05.553

Keywords

Animals; Cell Nucleolus/metabolism; Cell Nucleus/metabolism; Clone Cells; Coiled Bodies/metabolism; Cytoplasmic Structures/metabolism; Drosophila Proteins/metabolism; Drosophila melanogaster/cytology; Drosophila melanogaster/metabolism; Germ Cells/cytology; Germ Cells/metabolism; Heterochromatin/metabolism; Histones/metabolism; Mitosis; Models, Biological; Muscular Atrophy, Spinal/metabolism; Mutation/genetics; Oocytes/cytology; Oocytes/metabolism; Protein Transport; RNA, Small Nuclear/metabolism; RNA-Binding Proteins/metabolism

Significance

Annotations

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

DROME:SMN

part_of

GO:0071254: cytoplasmic U snRNP body

ECO:0000314: direct assay evidence used in manual assertion

C

  • part_of:(CL:0000026)|part_of:(CL:0000023)

Seeded From UniProt

complete

DROME:SMN

located_in

GO:0005737: cytoplasm

ECO:0000314: direct assay evidence used in manual assertion

C

  • part_of:(CL:0000477)|part_of:(CL:0000022)|part_of:(CL:0000722)

Seeded From UniProt

complete

DROME:SMN

involved_in

GO:0033962: P-body assembly

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:SMN

located_in

GO:0097504: Gemini of coiled bodies

ECO:0000314: direct assay evidence used in manual assertion

C

  • part_of:(CL:0000026)

Seeded From UniProt

complete

DROME:SMN

involved_in

GO:0051276: chromosome organization

ECO:0000315: mutant phenotype evidence used in manual assertion

P

  • occurs_in:(CL:0000026)|occurs_in:(CL:0000023)|occurs_in:(CL:0000477)

Seeded From UniProt

complete

DROME:SMN

involved_in

GO:1990194: cytoplasmic U snRNP body assembly

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VV74

involved_in

GO:1990194: cytoplasmic U snRNP body assembly

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VV74

part_of

GO:0005737: cytoplasm

ECO:0000314: direct assay evidence used in manual assertion

C

part_of:(CL:0000477)|part_of:(CL:0000022)|part_of:(CL:0000722)

Seeded From UniProt

complete

DROME:Q9VV74

part_of

GO:0071254: cytoplasmic U snRNP body

ECO:0000314: direct assay evidence used in manual assertion

C

part_of:(CL:0000026)|part_of:(CL:0000023)

Seeded From UniProt

complete

DROME:Q9VV74

involved_in

GO:0051276: chromosome organization

ECO:0000315: mutant phenotype evidence used in manual assertion

P

occurs_in:(CL:0000026)|occurs_in:(CL:0000023)|occurs_in:(CL:0000477)

Seeded From UniProt

complete

DROME:Q9VV74

involved_in

GO:0033962: cytoplasmic mRNA processing body assembly

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

DROME:Q9VV74

part_of

GO:0097504: Gemini of coiled bodies

ECO:0000314: direct assay evidence used in manual assertion

C

part_of:(CL:0000026)

Seeded From UniProt

complete

Notes

See also

References

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