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The SMN Tudor SIM-like domain is key to SmD1 and coilin interactions and to Cajal body biogenesis

Abstract: Cajal bodies (CBs) are nuclear organelles involved in the maturation of spliceosomal small nuclear ribonucleoproteins (snRNPs). They concentrate coilin, snRNPs and the survival motor neuron protein (SMN). Dysfunction of CB assembly occurs in spinal muscular atrophy (SMA). Here, we demonstrate that SMN is a SUMO1 target that has a small ubiquitin-related modifier (SUMO)-interacting motif (SIM)-like motif in the Tudor domain. The expression of SIM-like mutant constructs abolishes the interaction of SMN with the spliceosomal SmD1 (also known as SNRPD1), severely decreases SMN-coilin interaction and prevents CB assembly. Accordingly, the SMN SIM-like-mediated interactions are important for CB biogenesis and their dysfunction can be involved in SMA pathophysiology.

Otras publicaciones de la misma revista o congreso con autores/as de la Universidad de Cantabria

 Fuente: J Cell Sci. 2014 Mar 1;127(Pt 5):939-46

Editorial: Company of Biologists

 Año de publicación: 2014

Nº de páginas: 8

Tipo de publicación: Artículo de Revista

 DOI: 10.1242/jcs.138537

ISSN: 0021-9533,1477-9137

Autores/as

OLGA TAPIA MARTINEZ

LAFARGA, VANESA

ROCIO BENGOECHEA IBACETA

MIGUEL ANGEL LAFARGA COSCOJUELA

MARIA TERESA BERCIANO BLANCO