Lafora disease proteins malin and laforin are recruited to aggresomes in response to proteasomal impairment.
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A role for AGL ubiquitination in the glycogen storage disorders of Lafora and Cori's diseaseLafora progressive myoclonus epilepsy: NHLRC1 mutations affect glycogen metabolismLaforin is required for the functional activation of malin in endoplasmic reticulum stress resistance in neuronal cellsRecruitment of the oncoprotein v-ErbA to aggresomes.Increased endoplasmic reticulum stress and decreased proteasomal function in lafora disease models lacking the phosphatase laforinCo-chaperone CHIP stabilizes aggregate-prone malin, a ubiquitin ligase mutated in Lafora disease.The laforin-malin complex, involved in Lafora disease, promotes the incorporation of K63-linked ubiquitin chains into AMP-activated protein kinase beta subunits.Dimerization of the glucan phosphatase laforin requires the participation of cysteine 329.Frontier of epilepsy research - mTOR signaling pathway.PaCS is a novel cytoplasmic structure containing functional proteasome and inducible by cytokines/trophic factors.The laforin-malin complex negatively regulates glycogen synthesis by modulating cellular glucose uptake via glucose transporters.Ontogeny of Lafora bodies and neurocytoskeleton changes in Laforin-deficient mice.Laforin, a protein with many faces: glucan phosphatase, adapter protein, et alii.Deciphering the role of malin in the lafora progressive myoclonus epilepsyProtein tyrosine phosphatases: dual-specificity phosphatases in health and disease.Glycogen and its metabolism: some new developments and old themes.Deletions and missense mutations of EPM2A exacerbate unfolded protein response and apoptosis of neuronal cells induced by endoplasm reticulum stressHyperphosphorylation and aggregation of Tau in laforin-deficient mice, an animal model for Lafora disease.Novel mutation in the NHLRC1 gene in a Malian family with a severe phenotype of Lafora disease.Activation of serum/glucocorticoid-induced kinase 1 (SGK1) underlies increased glycogen levels, mTOR activation, and autophagy defects in Lafora disease.Disruption of normal cytoskeletal dynamics may play a key role in the pathogenesis of epilepsy.Lafora disease: insights into neurodegeneration from plant metabolism.Nucleolar aggresomes as counterparts of cytoplasmic aggresomes in proteotoxic stress. Proteasome inhibitors induce nuclear ribonucleoprotein inclusions that accumulate several key factors of neurodegenerative diseases and cancer.E3 ubiquitin ligases in protein quality control mechanism.Misfolded proteins recognition strategies of E3 ubiquitin ligases and neurodegenerative diseases.Emerging role of autophagy in pediatric neurodegenerative and neurometabolic diseases.Regulation of glycogen synthesis by the laforin-malin complex is modulated by the AMP-activated protein kinase pathway.Polyubiquitinated proteins, proteasome, and glycogen characterize the particle-rich cytoplasmic structure (PaCS) of neoplastic and fetal cells.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.PTG protein depletion rescues malin-deficient Lafora disease in mouse.Interdependence of laforin and malin proteins for their stability and functions could underlie the molecular basis of locus heterogeneity in Lafora disease.Proteasome-Rich PaCS as an Oncofetal UPS Structure Handling Cytosolic Polyubiquitinated Proteins. In Vivo Occurrence, in Vitro Induction, and Biological Role
P2860
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P2860
Lafora disease proteins malin and laforin are recruited to aggresomes in response to proteasomal impairment.
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年学术文章
@wuu
2007年学术文章
@zh-cn
2007年学术文章
@zh-hans
2007年学术文章
@zh-my
2007年学术文章
@zh-sg
2007年學術文章
@yue
2007年學術文章
@zh
2007年學術文章
@zh-hant
name
Lafora disease proteins malin ...... nse to proteasomal impairment.
@en
type
label
Lafora disease proteins malin ...... nse to proteasomal impairment.
@en
prefLabel
Lafora disease proteins malin ...... nse to proteasomal impairment.
@en
P2093
P2860
P356
P1476
Lafora disease proteins malin ...... nse to proteasomal impairment.
@en
P2093
Deepti Dubey
Kazuhiro Yamakawa
Shuchi Mittal
Subramaniam Ganesh
P2860
P304
P356
10.1093/HMG/DDM006
P577
2007-03-02T00:00:00Z