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Laforin

  • Reference work entry
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Encyclopedia of Signaling Molecules

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Synonyms

LAF-PTPase; EPM2A; MELF

Historical Background

Two groups searching for genes mutated in Lafora disease patients independently discovered the EPM2A (epilepsy, progressive myoclonic type 2A) gene, and the protein it encoded was aptly named laforin (Serratosa et al. 1995; Minassian et al. 1998). Laforin is a bimodular protein containing a dual specificity phosphatase (DSP) domain and a carbohydrate-binding module (CBM) (Fig. 1) (Minassian et al. 1998, Wang et al. 2002). Dual specificity phosphatases (DSPs) are a diverse group of phosphatases whose members dephosphorylate phosphoserine/phosphothreonine (pSer/pThr), phosphotyrosine (pTyr), phosphoinositols, ribo/deoxyribonucleotide 5′-triphosphates, pyrophosphate/triphosphate, or phosphoglucans. DSPs are members of the larger protein tyrosine phosphatase (PTP) superfamily that all utilize a cysteine-dependent mechanism to hydrolyze phosphoester bonds (Tonks 2006). This mechanism is dependent on the conserved CX5R active site motif...

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Authors and Affiliations

  1. Department of Molecular and Cellular Biochemistry, University of Kentucky College of Medicine, 741 S. Limestone, Lexington, KY, 40536, USA

    M. Kathryn Brewer, Amanda R. Sherwood & Matthew S. Gentry

  2. Department of Pharmaceutical Sciences, College of Pharmacy and Health Sciences, St. John’s University, Jamaica, NY, USA

    Vikas V. Dukhande

Authors
  1. M. Kathryn Brewer
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  2. Amanda R. Sherwood
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  3. Vikas V. Dukhande
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  4. Matthew S. Gentry
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Corresponding author

Correspondence to Matthew S. Gentry .

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Editors and Affiliations

  1. Department of Molecular Science and Technology, Ajou University, Suwon, Korea

    Sangdun Choi

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Kathryn Brewer, M., Sherwood, A.R., Dukhande, V.V., Gentry, M.S. (2018). Laforin. In: Choi, S. (eds) Encyclopedia of Signaling Molecules. Springer, Cham. https://doi.org/10.1007/978-3-319-67199-4_603

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