Muscle Nerve. 2018 Aug;58(2):286-292. doi: 10.1002/mus.26135. Epub 2018 Apr 23.

Quantification of lectin fluorescence in GNE myopathy muscle biopsies.


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 quantitative fluorescence method can accurately determine sialylation status of GNE myopathy muscle biopsies. This method is adaptable for expression of other membrane-associated muscle proteins, and may be of benefit for disorders in which therapeutic changes in expression are subtle and difficult to assess by other methods. Muscle Nerve 58: 286-292, 2018.


シアル酸に結合するSambucus nigra agglutinin (SNA) lectinと筋鞘膜のマーカーであるcaveolin-3 (CAV-3)抗体を用い、GNEミオパチー患者の筋肉を蛍光染色した。筋鞘膜でのSNA染色の蛍光発光は、GNEミオパチー患者においてコントロール症例より~50%減弱していた。本報告は、このような手技がGNEミオパチー患者の筋肉での低シアリル化を評価できることを示すとともに、他疾患においても筋鞘膜関連蛋白の発現量を解析し、治療効果判定に用いることができる可能性を示唆している。

PMID:  29603301