title: Assessing the Biological Activity of the Glucan Phosphatase Laforin.
abstract: Glucan phosphatases are a recently discovered family of enzymes that dephosphorylate either starch or glycogen and are essential for proper starch metabolism in plants and glycogen metabolism in humans. Mutations in the gene encoding the only human glucan phosphatase, laforin, result in the fatal, neurodegenerative, epilepsy known as Lafora disease. Here, we describe phosphatase assays to assess both generic laforin phosphatase activity and laforin's unique glycogen phosphatase activity.
authors: Department of Physiology, School of Medicine and Dentistry, University of Valencia and FIHCUV-INCLIVA, Valencia, Spain.|Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Jaime Roig 11, 46010, Valencia, Spain.
authors: Carlos
authors: C
authors: Romá-Mateo
authors: Department of Molecular and Cellular Biochemistry, Center for Structural Biology, University of Kentucky, Lexington, KY, USA.
authors: Madushi
authors: M
authors: Raththagala
authors: Department of Molecular and Cellular Biochemistry, Center for Structural Biology, University of Kentucky, Lexington, KY, USA.
authors: Mathew S
authors: MS
authors: Gentry
authors: Instituto de Biomedicina de Valencia, CSIC, Valencia, Spain. sanz@ibv.csic.es.|Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Jaime Roig 11, 46010, Valencia, Spain. sanz@ibv.csic.es.
authors: Pascual
authors: P
authors: Sanz
mesh_terms: D057075:Enzyme Assays / Q000379:methods*; D005452:Fluoresceins / Q000378:metabolism; D006003:Glycogen / Q000378:metabolism*; D006801:Humans; D020192:Lafora Disease / Q000201:enzymology* / Q000378:metabolism; D009596:Nitrophenols / Q000378:metabolism; D009943:Organophosphorus Compounds / Q000378:metabolism; D054558:Protein Tyrosine Phosphatases, Non-Receptor / Q000378:metabolism*; D013379:Substrate Specificity
chemical_list: C032079:3-O-methylfluorescein phosphate; D005452:Fluoresceins; D009596:Nitrophenols; D009943:Organophosphorus Compounds; C008644:nitrophenylphosphate; D006003:Glycogen; D054558:Protein Tyrosine Phosphatases, Non-Receptor; C114990:EPM2A protein, human
keywords: Dual-specificity phosphatase; Glycogen; Lafora disease