The ability to reliably quantify the relative degree of storage burden that results from lysosomal dysfunction is an important goal. Such measurements not only allow an assessment of different stages of disease progression, but also the assessment of therapeutic strategies. Although biochemical methods exist for doing this, retaining the anatomical integrity of tissue samples is an important consideration. This chapter provides practical methodological recommendations for achieving this goal in tissue sections, either by directly visualizing or staining the storage material, and subsequently quantifying it via image analysis.
Keywords: Assessing therapeutic efficacy; Autofluorescence; Confocal microscopy; Disease progression; Immunostaining; Lysosomal dysfunction; Quantitative analysis; Storage material accumulation; Thresholding image analysis.
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