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RESEARCH PRODUCT
Assessing the biological activity of the glucan phosphatase laforin
Carlos Romá-mateoMadushi RaththagalaPascual SanzMathew S. Gentrysubject
0301 basic medicinePhosphataseLafora diseaseArticleSubstrate SpecificityNitrophenols03 medical and health scienceschemistry.chemical_compound0302 clinical medicineOrganophosphorus CompoundsDual-specificity phosphatasemedicineHumansGlucanEnzyme Assayschemistry.chemical_classificationGlycogenbiologyfood and beveragesBiological activitymedicine.diseaseFluoresceinsProtein Tyrosine Phosphatases Non-Receptor030104 developmental biologyEnzymechemistryBiochemistryLafora Diseasebiology.proteinLaforin030217 neurology & neurosurgeryGlycogendescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2016-01-01 |