Validation of a Muscle-Specific Tissue Image Analysis Tool for Quantitative Assessment of Dystrophin Staining in Frozen Muscle Biopsies.

ABSTRACT:

Context.—

Duchenne muscular dystrophy is a rare, progressive, and fatal neuromuscular disease caused by dystrophin protein loss. Common investigational treatment approaches aim at increasing dystrophin expression in diseased muscle. Some clinical trials include assessments of novel dystrophin production as a surrogate biomarker of efficacy, which may predict a clinical benefit from treatment.

Objectives.—

To establish an immunofluorescent scanning and digital image analysis workflow that provides an objective approach for staining intensity assessment of the immunofluorescence dystrophin labeling and determination of the percentage of biomarker-positive fibers in muscle cryosections.

Design.—

Optimal and repeatable digital image capture was achieved by a rigorously qu

SUBMITTER: Aeffner F 

PROVIDER: S-EPMC6813837 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature