The phosphatase laforin crosses evolutionary boundaries and links carbohydrate metabolism to neuronal disease.

The phosphatase laforin crosses evolutionary boundaries and links carbohydrate metabolism to neuronal disease. Research Abstract Details 

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The phosphatase laforin crosses evolutionary boundaries and links carbohydrate metabolism to neuronal disease. Abstract Text:

Matthew S Gentry,Robert H Dowen,Carolyn A Worby,Seema Mattoo,Joseph R Ecker,Jack E Dixon,Matthew S Gentry,Robert H Dowen,Carolyn A Worby,Seema Mattoo,Joseph R Ecker,Jack E Dixon,

Lafora disease (LD) is a progressive myoclonic epilepsy resulting in severe neurodegeneration followed by death. A hallmark of LD is the accumulation of insoluble polyglucosans called Lafora bodies (LBs). LD is caused by mutations in the gene encoding the phosphatase laforin, which reportedly exists solely in vertebrates. We utilized a bioinformatics screen to identify laforin orthologues in five protists. These protists evolved from a progenitor red alga and synthesize an insoluble carbohydrate whose composition closely resembles LBs. Furthermore, we show that the kingdom Plantae, which lacks laforin, possesses a protein with laforin-like properties called starch excess 4 (SEX4). Mutations in the Arabidopsis thaliana SEX4 gene results in a starch excess phenotype reminiscent of LD. We demonstrate that Homo sapiens laforin complements the sex4 phenotype and propose that laforin and SEX4 are functional equivalents. Finally, we show that laforins and SEX4 dephosphorylate a complex carbohydrate and form the only family of phosphatases with this activity. These results provide a molecular explanation for the etiology of LD.

The phosphatase laforin crosses evolutionary boundaries and links carbohydrate metabolism to neuronal disease. Publishing Authors By Initials

MS Gentry,RH Dowen,CA Worby,S Mattoo,JR Ecker,JE Dixon,MS Gentry,RH Dowen,CA Worby,S Mattoo,JR Ecker,JE Dixon,

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The phosphatase laforin crosses evolutionary boundaries and links carbohydrate metabolism to neuronal disease. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: The Journal of cell biology

VOLUME: 178

Page Numbers: 477-88

Journal Abbreviation: J. Cell Biol.

ISSN: 0021-9525

DAY: 23

MONTH: 07

YEAR: 2007

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LANGUAGE: eng

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Grant and Affiliation Information for The phosphatase laforin crosses evolutionary boundaries and links carbohydrate metabolism to neuronal disease.

AFFILIATION: Department of Pharmacology, University of California, San Diego, La Jolla, CA 92093, USA.

Country: United States

AGENCY: United States NICHD

GRANT: T32 HD007203-23

ACRONYM: HD

MEDLINETA: J Cell Biol

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