Lafora bodies and neurological defects in malin-deficient mice correlate with impaired autophagy. - Wikidata - awgldk - TriplyDB

Lafora bodies and neurological defects in malin-deficient mice correlate with impaired autophagy.

Lafora bodies and neurological defects in malin-deficient mice correlate with impaired autophagy.

http://www.wikidata.org/entity/Q43864961

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Autophagy and neurodegeneration Role of autophagy in glycogen breakdown and its relevance to chloroquine myopathy Loss of GABAergic cortical neurons underlies the neuropathology of Lafora disease LAMP-2 deficiency leads to hippocampal dysfunction but normal clearance of neuronal substrates of chaperone-mediated autophagy in a mouse model for Danon disease Abnormal glycogen chain length pattern, not hyperphosphorylation, is critical in Lafora disease.Protein degradation and quality control in cells from laforin and malin knockout mice.Does abnormal glycogen structure contribute to increased susceptibility to seizures in epilepsy?Increased oxidative stress and impaired antioxidant response in Lafora disease.Dimerization of the glucan phosphatase laforin requires the participation of cysteine 329.Glycogen phosphomonoester distribution in mouse models of the progressive myoclonic epilepsy, Lafora disease.Regulation of autophagy by mTOR-dependent and mTOR-independent pathways: autophagy dysfunction in neurodegenerative diseases and therapeutic application of autophagy enhancers.Laforin, a protein with many faces: glucan phosphatase, adapter protein, et alii.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 Deleterious effects of neuronal accumulation of glycogen in flies and mice Neuronatin-mediated aberrant calcium signaling and endoplasmic reticulum stress underlie neuropathology in Lafora disease 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 Phenotype variations in Lafora progressive myoclonus epilepsy: possible involvement of genetic modifiers?Emerging role of autophagy in pediatric neurodegenerative and neurometabolic diseases.Autophagy and amino acid metabolism in the brain: implications for epilepsy.The role of autophagy in epileptogenesis and in epilepsy-induced neuronal alterations.Changing shapes of glycogen-autophagy nexus in neurons: perspective from a rare epilepsy.Glycogen phosphorylation and Lafora disease.miR-96 attenuates status epilepticus-induced brain injury by directly targeting Atg7 and Atg16L1.Enhanced sensitivity of laforin- and malin-deficient mice to the convulsant agent pentylenetetrazole Inflammation in Lafora Disease: Evolution with Disease Progression in Laforin and Malin Knock-out Mouse Models.Homeostasis of the astrocytic glutamate transporter GLT-1 is altered in mouse models of Lafora disease.Pharmacological Interventions to Ameliorate Neuropathological Symptoms in a Mouse Model of Lafora Disease.Loss of malin, but not laforin, results in compromised autophagic flux and proteasomal dysfunction in cells exposed to heat shock.The phosphatase activity of laforin is dispensable to rescue Epm2a-/- mice from Lafora disease.Sodium selenate treatment improves symptoms and seizure susceptibility in a malin-deficient mouse model of Lafora disease.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.4-Phenylbutyric acid and metformin decrease sensitivity to pentylenetetrazol-induced seizures in a malin knockout model of Lafora disease.Astrocytes: new players in progressive myoclonus epilepsy of Lafora type.Glycogen accumulation underlies neurodegeneration and autophagy impairment in Lafora disease.Interdependence of laforin and malin proteins for their stability and functions could underlie the molecular basis of locus heterogeneity in Lafora disease.SGK1 (glucose transport), dishevelled2 (wnt signaling), LC3/p62 (autophagy) and p53 (apoptosis) proteins are unaltered in Lafora disease.Lafora Disease Is an Inherited Metabolic Cardiomyopathy.

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Lafora bodies and neurological defects in malin-deficient mice correlate with impaired autophagy.

http://www.wikidata.org/entity/Q43864961

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2011 nî lūn-bûn

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2011年の論文

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2011年学术文章

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2011年学术文章

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2011年学术文章

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2011年学术文章

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2011年学术文章

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2011年学术文章

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2011年學術文章

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name

Lafora bodies and neurological ...... elate with impaired autophagy.

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Lafora bodies and neurological ...... elate with impaired autophagy.

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type

label

Lafora bodies and neurological ...... elate with impaired autophagy.

@en

Lafora bodies and neurological ...... elate with impaired autophagy.

@nl

prefLabel

Lafora bodies and neurological ...... elate with impaired autophagy.

@en

Lafora bodies and neurological ...... elate with impaired autophagy.

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Human Molecular Genetics

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Lafora bodies and neurological ...... elate with impaired autophagy.

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Ana M Garcia-Cabrero

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

Beatriz San Millán

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Carlos Romá-Mateo

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Carmen Aguado

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

Carmen Navarro

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Erwin Knecht

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Javier Gayarre

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Jose M Serratosa

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Lara Duran-Trio

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Lucía Juana-López

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P2860

Laforin, defective in the progressive myoclonus epilepsy of Lafora type, is a dual-specificity phosphatase associated with polyribosomes

A role for AGL ubiquitination in the glycogen storage disorders of Lafora and Cori's disease

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

Malin decreases glycogen accumulation by promoting the degradation of protein targeting to glycogen (PTG)

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

Laforin, the most common protein mutated in Lafora disease, regulates autophagy

LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing

Advances in genetics of juvenile myoclonic epilepsies

Laforin, a dual specificity phosphatase that dephosphorylates complex carbohydrates

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

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1521-1533

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scholarly article

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10.1093/HMG/DDR590

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7

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21

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Santiago Rodriguez de Córdoba

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2011-12-20T00:00:00Z

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22186026

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