A New Analytical Bench Assay for the Determination of Arylsulfatase A Activity Toward Galactosyl-3-Sulfate Ceramide: Implication for Metachromatic Leukodystrophy Diagnosis

• Published in: Analytical chemistry (Washington) Vol. 86; no. 1; pp. 473 - 481
• Main Authors: Morena, Francesco, di Girolamo, Ilaria, Emiliani, Carla, Gritti, Angela, Biffi, Alessandra, Martino, Sabata
• Format: Journal Article
• Language: English
• Published: United States American Chemical Society 07.01.2014
• Subjects: Analytical chemistry; analytical specificity; Animal models; Animals; arylsulfatase; Assaying; Cattle; ceramides; Cerebroside-Sulfatase - analysis; Cerebroside-Sulfatase - metabolism; colorimetry; Colorimetry - methods; Diagnosis; Effectiveness; enzymatic reactions; Enzyme Activation - physiology; Enzymes; Humans; hydrolysis; Leukodystrophy, Metachromatic - diagnosis; Leukodystrophy, Metachromatic - enzymology; Mathematical analysis; Medical services; Mice; Monitoring; Patients; spectrophotometers; Spectrophotometry - methods; Substrates; Sulfates; Sulfoglycosphingolipids - chemistry; Wavelengths

Here, we present the design and validation of a new assay for the diagnosis of metachromatic leukodystrophy. The method is highly specific, simple, reproducible, and straightforward. In our spectrophotometric method, the determination of arylsulfatase A (ARSA) activity toward the natural substrate,...