Quantification of lectin fluorescence in GNE myopathy muscle biopsies.

ABSTRACT:

Introduction

GNE myopathy is an adult-onset muscle disorder characterized by impaired sialylation of (muscle) glycans, detectable by lectin histochemistry. We describe a standardized method to quantify (lectin-) fluorescence in muscle sections, applicable for diagnosis and response to therapy for GNE myopathy.

Methods

Muscle sections were fluorescently labeled with the sialic acid-binding Sambucus nigra agglutinin (SNA) lectin and antibodies to sarcolemma residence protein caveolin-3 (CAV-3). Entire tissue sections were imaged in tiles and fluorescence was quantified.

Results

SNA fluorescence co-localizing with CAV-3 was ∼50% decreased in GNE myopathy biopsies compared with muscle-matched controls, confirming previous qualitative results.

Discussion

This quanti

SUBMITTER: Leoyklang P 

PROVIDER: S-EPMC6105422 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature