Dystrophin quantification: Biological and translational research implications.

ABSTRACT:

Objective

We formed a multi-institution collaboration in order to compare dystrophin quantification methods, reach a consensus on the most reliable method, and report its biological significance in the context of clinical trials.

Methods

Five laboratories with expertise in dystrophin quantification performed a data-driven comparative analysis of a single reference set of normal and dystrophinopathy muscle biopsies using quantitative immunohistochemistry and Western blotting. We developed standardized protocols and assessed inter- and intralaboratory variability over a wide range of dystrophin expression levels.

Results

Results from the different laboratories were highly concordant with minimal inter- and intralaboratory variability, particularly with quantitative immu

SUBMITTER: Anthony K 

PROVIDER: S-EPMC4248450 | biostudies-literature | 2014 Nov

REPOSITORIES: biostudies-literature