|ZFIN ID: ZDB-PUB-061205-15|
Loss of selenoprotein N function causes disruption of muscle architecture in the zebrafish embryo
Deniziak, M., Thisse, C., Rederstorff, M., Hindelang, C., Thisse, B., and Lescure, A.
|Source:||Experimental cell research 313(1): 156-167 (Journal)|
|Registered Authors:||Lescure, Alain, Thisse, Bernard, Thisse, Christine|
|Keywords:||Selenoprotein N, Selenocysteine, Congenital muscular dystrophy, Zebrafish, Muscle, Myoseptum|
|PubMed:||17123513 Full text @ Exp. Cell Res.|
Deniziak, M., Thisse, C., Rederstorff, M., Hindelang, C., Thisse, B., and Lescure, A. (2007) Loss of selenoprotein N function causes disruption of muscle architecture in the zebrafish embryo. Experimental cell research. 313(1):156-167.
ABSTRACTMutations in the gene coding for selenoprotein N (SelN), a selenium containing protein of unknown function, cause different forms of congenital muscular dystrophy in humans. These muscular diseases are characterized by early onset of hypotonia which predominantly affect in axial muscles. We used zebrafish as a model system to understand the function of SelN in muscle formation during embryogenesis. Zebrafish SelN is highly homologous to its human counterpart and amino acids corresponding to the mutated positions in human muscle diseases are conserved in the zebrafish protein. The sepn1 gene is highly expressed in the somites and notochord during early development. Inhibition of the sepn1 gene by injection of antisense morpholinos does not alter the fate of the muscular tissue, but causes muscle architecture disorganization and greatly reduced motility. Ultrastructural analysis of the myotomes reveals defects in muscle sarcomeric organization and in myofibers attachment, as well as altered myoseptum integrity. These studies demonstrate the important role of SelN for muscle organization during early development. Moreover, alteration of myofibrils architecture and tendon-like structure in embryo deficient for SelN function provide new insights into the pathological mechanism of SelN-related myopathy.