Abstract
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. (C) 2000 Federation of European Biochemical Societies. Published by Elsevier Science B.V. All rights reserved.
| Original language | English |
|---|---|
| Pages (from-to) | 13 - 18 |
| Number of pages | 6 |
| Journal | FEBS Letters |
| Volume | 482 |
| Issue number | 1-2 |
| Publication status | Published - 29 Sept 2000 |