Assessment of Lectin Staining Biomarkers using a Murine Model of GNE Myopathy

TL;DR

Researchers tested lectin biomarkers in a mouse model of GNE myopathy to detect sialic acid levels, finding Peanut Agglutinin as a potential tool for identifying hyposialylation.

Contribution

Identified Peanut Agglutinin as a promising lectin for detecting hyposialylation in murine GNE myopathy tissues.

Findings

No lectins effectively differentiated Gne M743T/M743T from wild type tissues.

Peanut Agglutinin showed differential binding in sialidase-treated tissues, indicating potential for detecting hyposialylation.

Abstract

GNE myopathy (GNEM) is a rare myopathy caused by mutations in the UDP-GlcNAc epimerase/ManNAc-6 kinase
(GNE)
gene, which reduce sialic acid (SA) biosynthesis and impair muscle through unclear mechanisms. As development of SA-restoring GNEM gene therapies is underway, it is essential to develop SA-detecting biomarkers in preclinically-relevant murine tissues. Here, we assess skeletal muscle staining of the

Gne
M743T/M743T

GNEM model with four sialylation-detecting lectins. While no tested lectins could effectively differentiate between

Gne
M743T/M743T

and wild type tissues, Peanut Agglutinin (PNA) showed differential binding in tissues with and without SA-removing sialidase treatment, indicating its promise in detecting hyposialylation in murine tissues.

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