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XB-ART-17970
EMBO J July 15, 1996; 15 (14): 3555-65.

A novel nuclear structure containing the survival of motor neurons protein.

Liu Q , Dreyfuss G .


Abstract
Spinal muscular atrophy (SMA) is a common, often fatal, autosomal recessive disease leading to progressive muscle wasting and paralysis as a result of degeneration of anterior horn cells of the spinal cord. A gene termed survival of motor neurons (SMN), at 5q13, has been identified as the determining gene of SMA (Lefebvre et al., 1995). The SMN gene is deleted in > 98% of SMA patients, but the function of the SMN protein is unknown. In searching for hnRNP-interacting proteins we found that SMN interacts with the RGG box region of hnRNP U, with itself, with fibrillarin and with several novel proteins. We have produced monoclonal antibodies to the SMN protein, and we report here on its striking cellular localization pattern. Immunolocalization studies using SMN monoclonal antibodies show several intense dots in HeLa cell nuclei. These structures are similar in number (2-6) and size (0.1-1.0 micron) to coiled bodies, and frequently are found near or associated with coiled bodies. We term these prominent nuclear structures gems, for Gemini of coiled bodies.

PubMed ID: 8670859
PMC ID: PMC451956
Article link:


Species referenced: Xenopus laevis
Genes referenced: acta2 hnrnpc smn1 tbx2

References [+] :
Andrade, Immunocytochemical analysis of the coiled body in the cell cycle and during cell proliferation. 1993, Pubmed