Evaluation of the Lipid-binding Properties of Recombinant Dystrophin Spectrin-like Repeat Domains R1-3

J Neuromuscul Dis. 2021;8(4):489-494. doi: 10.3233/JND-200622.

Abstract

Recombinant micro-dystrophin genes are designed to treat Duchenne muscular dystrophy (DMD) by retaining dystrophin domains believed to play key functional roles while fitting the packaging capacity of adeno-associated virus vectors. Domains R1-3 are important for muscle force generation and for association with the sarcolemma, but the nature of this interaction is not fully understood. We measured lipid-binding affinity of 3 peptides containing different spectrin-like repeat modules (R1-3; R1-2; and R1, 2, 22). Lipid-binding affinity was highest with R1-3, suggesting that the complete R1-R3 region could be beneficial and should be considered for inclusion in micro-dystrophin constructs.

Keywords: Lipid binding; dystrophin; spectrin-like repeats.

Publication types

  • Letter

MeSH terms

  • Dystrophin / genetics*
  • Humans
  • Lipids / genetics
  • Muscular Dystrophy, Duchenne / genetics*
  • Sarcolemma / genetics
  • Spectrin / genetics*

Substances

  • DMD protein, human
  • Dystrophin
  • Lipids
  • Spectrin