Spinal muscular atrophy disrupts the interaction of ZPR1 with the SMN protein

Nature Cell Biology

Volumn 3, Issue 4, 2001, Pages 376-383

  Gangwani, Laxman ^a   Mikrut, Monique ^a   Theroux, Steven ^b   Sharma, Manoj ^a,c   Davis, Roger J ^a,d  

^a UNIVERSITY OF MASSACHUSETTS MEDICAL SCHOOL   (United States)

^b ASSUMPTION COLLEGE   (United States)

^c Strategic BioSolutions Inc   (United States)

^d HOWARD HUGHES MEDICAL INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

GENE PRODUCT; SURVIVAL MOTOR NEURON PROTEIN; UNCLASSIFIED DRUG; ZINC FINGER PROTEIN;

ANIMAL CELL; ARTICLE; CELL NUCLEUS; CELL NUCLEUS INCLUSION BODY; CELLULAR DISTRIBUTION; COILED BODY; CONTROLLED STUDY; CYTOPLASM; GENE MUTATION; HUMAN; HUMAN CELL; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; PROTEIN PROTEIN INTERACTION; SERUM; SPINAL MUSCULAR ATROPHY; WERDNIG HOFFMANN DISEASE;

ALTERNATIVE SPLICING; ANIMALS; CARRIER PROTEINS; CELL NUCLEUS; CERCOPITHECUS AETHIOPS; COS CELLS; CYCLIC AMP RESPONSE ELEMENT-BINDING PROTEIN; CYTOPLASM; HELA CELLS; HUMANS; MUSCULAR ATROPHY, SPINAL; NERVE TISSUE PROTEINS; RNA PRECURSORS; RNA-BINDING PROTEINS; ZINC FINGERS;

ANIMALIA;

EID: 0035074529     PISSN: 14657392     EISSN: None     Source Type: Journal    
DOI: 10.1038/35070059     Document Type: Article

References (44)

1. An update of the mutation spectrum of the survival motor neuron gene (SMN1) in autosomal recessive spinal muscular atrophy (SMA)
2. Identification and characterization of a spinal muscular atrophy- determining gene
3. Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryos
4. The Caenorhabditis elegans orthologue of the human gene responsible for spinal muscular atrophy is a maternal product critical for germline maturation and embryonic viability
5. A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2
6. A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy
7. Nuclear targeting defect of SMN lacking the C-terminus in a mouse model of spinal muscular atrophy
8. A mouse model for spinal muscular atrophy
9. -/- mice and results in a mouse with spinal muscular atrophy
10. A novel nuclear structure containing the survival of motor neurons protein
11. Coiled bodies and gems: Janus or gemini?
12. The spinal muscular atrophy disease gene product, SMN: A link between snRNP biogenesis and the Cajal (coiled) body
13. The relationship between SMN, the spinal muscular atrophy protein, and nuclear coiled bodies in differentiated tissues and cultured cells
14. The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1, are in a complex with spliceosomal snRNP proteins
15. The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis
16. A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing
17. Subcellular localization and axonal transport of the survival motor neuron (SMN) protein in the developing rat spinal cord
18. The survival motor neuron protein in spinal muscular atrophy
19. Correlation between severity and SMN protein level in spinal muscular atrophy
20. Binding of zinc-finger protein ZPR1 to the epidermal growth factor receptor
21. The cytoplasmic zinc-finger protein ZPR1 accumulates in the nucleolus of proliferating cells
22. Interaction of ZPR1 with translation elongation factor-1α in proliferating cells
23. The genome sequence of Drosophila melanogaster
24. The C. elegans sequencing consortium. Genome sequence of the nematode C. elegans: A platform for investigating biology
25. The RNA-binding properties of SMN: Deletion analysis of the zebrafish orthologue defines domains conserved in evolution
26. Characterization of the Schizosaccharomyces pombe orthologue of the human survival motor neuron (SMN) protein
27. Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems
28. SMN oligomerization defect correlates with spinal muscular atrophy severity
29. Gemin4. A novel component of the smn complex that is found in both gems and nucleoli
30. Synergistic anti-apoptotic activity between Bcl-2 and SMN implicated in spinal muscular atrophy
31. Identification of survival motor neuron as a transcriptional activator-binding protein
32. The survival motor neuron protein interacts with the transactivator FUSE binding protein from human fetal brain
33. A role for polyproline motifs in the spinal muscular atrophy protein SMN. Profilins bind to and colocalize with smn in nuclear gems
34. Does the survival motor neuron protein (SMN) interact with Bcl-2?
35. Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of spliceosomal Sm proteins
36. SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins
37. Assembly of the nuclear transcription and processing machinery: Cajal bodies (coiled bodies) and transcriptosomes
38. Like attracts like: Getting RNA processing together in the nucleus
39. Nuclear organization of pre-mRNA splicing factors
40. Heterogeneity of subcellular localization and electrophoretic mobility of survival motor neuron (SMN) protein in mammalian neural cells and tissues
41. Immunocytochemical analysis of the coiled body in the cell cycle and during cell proliferation
42. Gel electrophoretic isolation of splicing complexes containing U1 small nuclear ribonucleoprotein particles