| Primary Identifier | IPR045204 | Type | Domain |
| Short Name | DSP_laforin-like |
| description | Proteins containing this domain include vertebrate dual specificity protein phosphatase Laforin and plant starch excess4 (SEX4). Laforin (encoded by the EPM2A gene) is a dual-specificity phosphatase that dephosphorylates complex carbohydrates. Mutations in the EPM2A gene cause Lafora disease (LD), a fatal autosomal recessive neurodegenerative disorder characterised by the presence of progressive neurological deterioration, myoclonus, and epilepsy []. Pathologically, LD is characterised by distinctive polyglucosans, which are formations of abnormal glycogen []. Laforin prevents LD by at least two mechanisms: by preventing hyperphosphorylation of glycogen by dephosphorylating it, allowing proper glycogen formation, and by promoting the ubiquitination of proteins involved in glycogen metabolism via its interaction with malin. Laforin contains an N-terminal CBM20 (carbohydrate-binding module, family 20) domain and a C-terminal catalytic dual specificity phosphatase (DSP) domain [].Plant SEX4 (also known as DSP4) regulates starch metabolism by selectively dephosphorylating glucose moieties within starch glucan chains. It contains an N-terminal catalytic DSP domain and a C-terminal Early (E) set domain []. |
