Faculty Opinions recommendation of Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein.

2002 ◽  
Author(s):  
Angus Lamond
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Cajal Bodies

scholarly journals Small-molecule flunarizine increases SMN protein in nuclear Cajal bodies and motor function in a mouse model of spinal muscular atrophy

2018 ◽  
Vol 8 (1) ◽  
Author(s):  
Delphine Sapaly ◽  
Matthieu Dos Santos ◽  
Perrine Delers ◽  
Olivier Biondi ◽  
Gwendoline Quérol ◽  
...  
Keyword(s):  
Mouse Model ◽  
Spinal Muscular Atrophy ◽  
Motor Function ◽  
Small Molecule ◽  
Muscular Atrophy ◽  
Cajal Bodies ◽  
Smn Protein

scholarly journals Cellular bases of the RNA metabolism dysfunction in motor neurons of a murine model of spinal muscular atrophy: Role of Cajal bodies and the nucleolus

2017 ◽  
Vol 108 ◽  
pp. 83-99 ◽  
Author(s):  
Olga Tapia ◽  
Josep Oriol Narcís ◽  
Javier Riancho ◽  
Olga Tarabal ◽  
Lídia Piedrafita ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neurons ◽  
Murine Model ◽  
Muscular Atrophy ◽  
Rna Metabolism ◽  
Cajal Bodies

scholarly journals The Spinal Muscular Atrophy Disease Gene Product, Smn

1999 ◽  
Vol 147 (4) ◽  
pp. 715-728 ◽  
Author(s):  
Teresa Carvalho ◽  
Fátima Almeida ◽  
Alexandre Calapez ◽  
Miguel Lafarga ◽  
Maria T. Berciano ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Gene Product ◽  
Disease Gene ◽  
Cultured Cells ◽  
Muscular Atrophy ◽  
Cajal Body ◽  
Cajal Bodies ◽  
Proliferating Cells ◽  
Sm Proteins ◽  
Coiled Bodies

The spliceosomal snRNAs U1, U2, U4, and U5 are synthesized in the nucleus, exported to the cytoplasm to assemble with Sm proteins, and reimported to the nucleus as ribonucleoprotein particles. Recently, two novel proteins involved in biogenesis of small nuclear ribonucleoproteins (snRNPs) were identified, the Spinal muscular atrophy disease gene product (SMN) and its associated protein SIP1. It was previously reported that in HeLa cells, SMN and SIP1 form discrete foci located next to Cajal (coiled) bodies, the so-called “gemini of coiled bodies” or “gems.” An intriguing feature of gems is that they do not appear to contain snRNPs. Here we show that gems are present in a variable but small proportion of rapidly proliferating cells in culture. In the vast majority of cultured cells and in all primary neurons analyzed, SMN and SIP1 colocalize precisely with snRNPs in the Cajal body. The presence of SMN and SIP1 in Cajal bodies is confirmed by immunoelectron microscopy and by microinjection of antibodies that interfere with the integrity of the structure. The association of SMN with snRNPs and coilin persists during cell division, but at the end of mitosis there is a lag period between assembly of new Cajal bodies in the nucleus and detection of SMN in these structures, suggesting that SMN is targeted to preformed Cajal bodies. Finally, treatment of cells with leptomycin B (a drug that blocks export of U snRNAs to the cytoplasm and consequently import of new snRNPs into the nucleus) is shown to deplete snRNPs (but not SMN or SIP1) from the Cajal body. This suggests that snRNPs flow through the Cajal body during their biogenesis pathway.

Reorganization of Cajal bodies and nucleolar targeting of coilin in motor neurons of type I spinal muscular atrophy

2012 ◽  
Vol 137 (5) ◽  
pp. 657-667 ◽  
Author(s):  
Olga Tapia ◽  
Rocío Bengoechea ◽  
Ana Palanca ◽  
Rosa Arteaga ◽  
J. Fernando Val-Bernal ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neurons ◽  
Muscular Atrophy ◽  
Cajal Bodies ◽  
Type I

scholarly journals The loss of the snoRNP chaperone Nopp140 from Cajal bodies of patient fibroblasts correlates with the severity of spinal muscular atrophy

2009 ◽  
Vol 18 (7) ◽  
pp. 1181-1189 ◽  
Author(s):  
B. Renvoise ◽  
S. Colasse ◽  
P. Burlet ◽  
L. Viollet ◽  
U. T. Meier ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Cajal Bodies
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