Glycogen hyperphosphorylation underlies lafora body formation. - Wikidata - wikimedia - TriplyDB

Glycogen hyperphosphorylation underlies lafora body formation.

Glycogen hyperphosphorylation underlies lafora body formation.

http://www.wikidata.org/entity/Q42815700

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Lafora progressive myoclonus epilepsy: NHLRC1 mutations affect glycogen metabolism Laforin is required for the functional activation of malin in endoplasmic reticulum stress resistance in neuronal cells Myocardial autophagic energy stress responses--macroautophagy, mitophagy, and glycophagy Early-onset Lafora body disease PTG depletion removes Lafora bodies and rescues the fatal epilepsy of Lafora disease Deficiency of a glycogen synthase-associated protein, Epm2aip1, causes decreased glycogen synthesis and hepatic insulin resistance Dimeric quaternary structure of human laforin.Abnormal glycogen chain length pattern, not hyperphosphorylation, is critical in Lafora disease.Laforin, a dual specificity phosphatase involved in Lafora disease, is present mainly as monomeric form with full phosphatase activity Progress and problems in muscle glycogenoses Glycogen phosphomonoester distribution in mouse models of the progressive myoclonic epilepsy, Lafora disease.Laforin, a dual-specificity phosphatase involved in Lafora disease, is phosphorylated at Ser25 by AMP-activated protein kinase.Laforin, a protein with many faces: glucan phosphatase, adapter protein, et alii.Neurodegeneration and functional impairments associated with glycogen synthase accumulation in a mouse model of Lafora disease Increased laforin and laforin binding to glycogen underlie Lafora body formation in malin-deficient Lafora disease.Deciphering the role of malin in the lafora progressive myoclonus epilepsy Mechanistic Insights into Glucan Phosphatase Activity against Polyglucan Substrates Neuronatin-mediated aberrant calcium signaling and endoplasmic reticulum stress underlie neuropathology in Lafora disease Laforin prevents stress-induced polyglucosan body formation and Lafora disease progression in neurons.Glycogen and its metabolism: some new developments and old themes.Activation of serum/glucocorticoid-induced kinase 1 (SGK1) underlies increased glycogen levels, mTOR activation, and autophagy defects in Lafora disease.Laforin-malin complex degrades polyglucosan bodies in concert with glycogen debranching enzyme and brain isoform glycogen phosphorylase Neuromuscular disorders of glycogen metabolism.Broad spectrum of hepatocyte inclusions in humans, animals, and experimental models.Changing shapes of glycogen-autophagy nexus in neurons: perspective from a rare epilepsy.Glycogen phosphorylation and Lafora disease.Incorporation of phosphate into glycogen by glycogen synthase Enhanced sensitivity of laforin- and malin-deficient mice to the convulsant agent pentylenetetrazole Lafora bodies in skeletal muscle are fiber type specific.Pharmacological Interventions to Ameliorate Neuropathological Symptoms in a Mouse Model of Lafora Disease.Phosphorylation prevents polyglucosan transport in Lafora disease.Laforin and malin knockout mice have normal glucose disposal and insulin sensitivity.Lafora bodies and neurological defects in malin-deficient mice correlate with impaired autophagy.The phosphatase activity of laforin is dispensable to rescue Epm2a-/- mice from Lafora disease.Mapping of Purkinje neuron loss and polyglucosan body accumulation in hereditary cerebellar degeneration in Scottish terriers.Suppression of leptin signaling reduces polyglucosan inclusions and seizure susceptibility in a mouse model for Lafora disease.PTG protein depletion rescues malin-deficient Lafora disease in mouse.Dysfunctions in endosomal-lysosomal and autophagy pathways underlie neuropathology in a mouse model for Lafora disease.Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase.Interdependence of laforin and malin proteins for their stability and functions could underlie the molecular basis of locus heterogeneity in Lafora disease.

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P2860

Glycogen hyperphosphorylation underlies lafora body formation.

http://www.wikidata.org/entity/Q42815700

description

2010 nî lūn-bûn

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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2010年论文

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2010年论文

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name

Glycogen hyperphosphorylation underlies lafora body formation.

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Glycogen hyperphosphorylation underlies lafora body formation.

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type

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Glycogen hyperphosphorylation underlies lafora body formation.

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Glycogen hyperphosphorylation underlies lafora body formation.

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Glycogen hyperphosphorylation underlies lafora body formation.

@en

Glycogen hyperphosphorylation underlies lafora body formation.

@nl

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P1433

Annals of Neurology

P1476

Glycogen hyperphosphorylation underlies lafora body formation.

@en

P2093

Alessandra Ruggieri

http://www.w3.org/2001/XMLSchema#string

Alexander P Kameka

http://www.w3.org/2001/XMLSchema#string

Arman G Draginov

http://www.w3.org/2001/XMLSchema#string

Berge A Minassian

http://www.w3.org/2001/XMLSchema#string

Cameron A Ackerley

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Jean-Marie Girard

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Julie Turnbull

http://www.w3.org/2001/XMLSchema#string

Nela Pencea

http://www.w3.org/2001/XMLSchema#string

Peixiang Wang

http://www.w3.org/2001/XMLSchema#string

Tony J Wang

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P2860

Glycogen and its Metabolism

Laforin, the dual-phosphatase responsible for Lafora disease, interacts with R5 (PTG), a regulatory subunit of protein phosphatase-1 that enhances glycogen accumulation

Insights into Lafora disease: malin is an E3 ubiquitin ligase that ubiquitinates and promotes the degradation of laforin.

Atypical ubiquitin chains: new molecular signals. 'Protein Modifications: Beyond the Usual Suspects' review series

Laforin, a dual specificity phosphatase that dephosphorylates complex carbohydrates

Characterization of the human P-type 6-phosphofructo-1-kinase gene promoter in neural cell lines

Targeted disruption of the Epm2a gene causes formation of Lafora inclusion bodies, neurodegeneration, ataxia, myoclonus epilepsy and impaired behavioral response in mice

A highly efficient recombineering-based method for generating conditional knockout mutations

AMP-activated protein kinase phosphorylates R5/PTG, the glycogen targeting subunit of the R5/PTG-protein phosphatase 1 holoenzyme, and accelerates its down-regulation by the laforin-malin complex

Laforin is a glycogen phosphatase, deficiency of which leads to elevated phosphorylation of glycogen in vivo.

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925-933

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scholarly article

P356

10.1002/ANA.22156

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P433

6

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P478

68

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2010-12-01T00:00:00Z

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47792488

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21077101

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