Dystrophin R16/17-syntrophin PDZ fusion protein restores sarcolemmal nNOSμ.

ABSTRACT:

Background

Loss of sarcolemmal nNOSμ is a common manifestation in a wide variety of muscle diseases and contributes to the dysregulation of multiple muscle activities. Given the critical role sarcolemmal nNOSμ plays in muscle, restoration of sarcolemmal nNOSμ should be considered as an important therapeutic goal.

Methods

nNOSμ is anchored to the sarcolemma by dystrophin spectrin-like repeats 16 and 17 (R16/17) and the syntrophin PDZ domain (Syn PDZ). To develop a strategy that can independently restore sarcolemmal nNOSμ, we engineered an R16/17-Syn PDZ fusion construct and tested whether this construct alone is sufficient to anchor nNOSμ to the sarcolemma in three different mouse models of Duchenne muscular dystrophy (DMD).

Results

Membrane-associated nNOSμ is complet

SUBMITTER: Patel A 

PROVIDER: S-EPMC6251231 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature