Conservation of components of the dystrophin complex in Drosophila 1

FEBS Letters

Roberts, Roland G¹  Greener, Marc J¹ 

[1] Division of Medical and Molecular Genetics, 8th Floor Guy's Tower, Guy's King's and St. Thomas’ Medical School, London SE1 9RT, UK

关键词: Dystrophin;    Duchenne muscular dystrophy;    Dystroglycan;    Dystrobrevin;    Syntrophin;    Sarcoglycan;    Limb-girdle muscular dystrophy;    Drosophila melanogaster;   

DOI  :  10.1016/S0014-5793(00)02018-4

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

Defects in the dystrophin complex (DC) underlie several human genetic disorders, but our dissection of its function is complicated by potential redundancy of the multiple vertebrate isoforms of most DC components. We here complete our previous description of Drosophila dystrophin, and show that the fly retains all essential components of the DC, but with substantially less diversity. Seventeen known human components (three dystrophin-related proteins, two dystrobrevins, five sarcoglycans, five syntrophins, one dystroglycan and one sarcospan) appear to be reduced to eight in Drosophila (one, one, three, two, one and none, respectively). The simplicity of this system recommends it as a model for its human counterpart.

【 授权许可】

Unknown