ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1. - Wikidata - awgldk - TriplyDB

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.

http://www.wikidata.org/entity/Q38947838

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The roles of ASK family proteins in stress responses and diseases Roquin-2 promotes ubiquitin-mediated degradation of ASK1 to regulate stress responses Ubiquilin and p97/VCP bind erasin, forming a complex involved in ERAD CHIP-dependent termination of MEKK2 regulates temporal ERK activation required for proper hyperosmotic response Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradation XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy Adaptive preconditioning in neurological diseases – therapeutic insights from proteostatic perturbations The Unfolded Protein Response and the Role of Protein Disulfide Isomerase in Neurodegeneration Endoplasmic Reticulum Stress Interacts With Inflammation in Human Diseases Molecular motor proteins and amyotrophic lateral sclerosis Neuronal endoplasmic reticulum stress in axon injury and neurodegeneration Xenobiotic perturbation of ER stress and the unfolded protein response Therapeutic targets in the ASK1-dependent stress signaling pathways An ALS-linked mutant SOD1 produces a locomotor defect associated with aggregation and synaptic dysfunction when expressed in neurons of Caenorhabditis elegans Functional contribution of the transcription factor ATF4 to the pathogenesis of amyotrophic lateral sclerosis Late-onset of spinal neurodegeneration in knock-in mice expressing a mutant BiP Divergent effects of PERK and IRE1 signaling on cell viability Review: Modulating the unfolded protein response to prevent neurodegeneration and enhance memory The complex molecular biology of amyotrophic lateral sclerosis (ALS)Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosis The critical role of membralin in postnatal motor neuron survival and disease Major histocompatibility complex class I molecules protect motor neurons from astrocyte-induced toxicity in amyotrophic lateral sclerosis.Palmitoylation of superoxide dismutase 1 (SOD1) is increased for familial amyotrophic lateral sclerosis-linked SOD1 mutants The amyotrophic lateral sclerosis 8 protein, VAP, is required for ER protein quality control A random set scoring model for prioritization of disease candidate genes using protein complexes and data-mining of GeneRIF, OMIM and PubMed records.Accumulation of wildtype and ALS-linked mutated VAPB impairs activity of the proteasome.Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS.An in vitro screening cascade to identify neuroprotective antioxidants in ALS Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCP.Genetic Correction of SOD1 Mutant iPSCs Reveals ERK and JNK Activated AP1 as a Driver of Neurodegeneration in Amyotrophic Lateral Sclerosis.Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia Continuum Global gene expression profiling of somatic motor neuron populations with different vulnerability identify molecules and pathways of degeneration and protection Polymorphic cis- and trans-regulation of human gene expression.Endoplasmic reticulum stress and inflammation in the central nervous system CIIA prevents SOD1(G93A)-induced cytotoxicity by blocking ASK1-mediated signaling Apoptosis signal-regulating kinase 1 mediates MPTP toxicity and regulates glial activation.Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress.Guanabenz, which enhances the unfolded protein response, ameliorates mutant SOD1-induced amyotrophic lateral sclerosis.

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P2860

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.

http://www.wikidata.org/entity/Q38947838

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2008 nî lūn-bûn

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ALS-linked mutant SOD1 induces ...... n death by targeting Derlin-1.

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ALS-linked mutant SOD1 induces ...... n death by targeting Derlin-1.

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ALS-linked mutant SOD1 induces ...... n death by targeting Derlin-1.

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ALS-linked mutant SOD1 induces ...... on death by targeting Derlin-1

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Atsushi Matsuzawa

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Atsushi Nagai

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Hisae Kadowaki

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Hisashi Fukutomi

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Takanori Yokota

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Takeshi Maruyama

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P2860

The tumor autocrine motility factor receptor, gp78, is a ubiquitin protein ligase implicated in degradation from the endoplasmic reticulum

A membrane protein required for dislocation of misfolded proteins from the ER

A membrane protein complex mediates retro-translocation from the ER lumen into the cytosol

ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats

Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis

Simultaneous induction of the four subunits of the TRAP complex by ER stress accelerates ER degradation

Derlin-2 and Derlin-3 are regulated by the mammalian unfolded protein response and are required for ER-associated degradation

Recruitment of the p97 ATPase and ubiquitin ligases to the site of retrotranslocation at the endoplasmic reticulum membrane

Multiprotein complexes that link dislocation, ubiquitination, and extraction of misfolded proteins from the endoplasmic reticulum membrane.

Replication of a cytopathic strain of bovine viral diarrhea virus activates PERK and induces endoplasmic reticulum stress-mediated apoptosis of MDBK cells

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10.1101/GAD.1640108

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11

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22

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Hidenori Ichijo

Hideki Nishitoh

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2008-06-01T00:00:00Z

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51396231

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18519638

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amyotrophic lateral sclerosis

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2418582

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