Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.
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Molecular basis of α1-antitrypsin deficiency revealed by the structure of a domain-swapped trimerGenes and chronic obstructive pulmonary diseaseEndoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpinsIs there a therapeutic role for selenium in alpha-1 antitrypsin deficiency?Capitalizing on the autophagic response for treatment of liver disease caused by alpha-1-antitrypsin deficiency and other genetic diseasesAlpha-1-antitrypsin deficiencyMechanisms of protein-folding diseases at a glanceFluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiencyBarriers to inhaled gene therapy of obstructive lung diseases: A reviewNew Concepts in Alpha-1 Antitrypsin Deficiency Disease Mechanisms.ER stress in Alzheimer's disease: a novel neuronal trigger for inflammation and Alzheimer's pathology.Z α1-antitrypsin confers a proinflammatory phenotype that contributes to chronic obstructive pulmonary diseaseBiP availability distinguishes states of homeostasis and stress in the endoplasmic reticulum of living cells.A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnoverA genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency.Defining the mechanism of polymerization in the serpinopathies.Targeting intracellular degradation pathways for treatment of liver disease caused by α1-antitrypsin deficiencyHepatic progenitor cell proliferation and liver injury in α-1-antitrypsin deficiency.Proinsulin misfolding and diabetes: mutant INS gene-induced diabetes of youth.Protein misfolding and the serpinopathies.ERManI is a target of miR-125b and promotes transformation phenotypes in hepatocellular carcinoma (HCC)Z α-1 antitrypsin deficiency and the endoplasmic reticulum stress response.Targeted protein destabilization reveals an estrogen-mediated ER stress response.Mutant myocilin nonsecretion in vivo is not sufficient to cause glaucoma.Role of Drosophila EDEMs in the degradation of the alpha-1-antitrypsin Z variant.Therapeutic targeting of misfolding and conformational change in α1-antitrypsin deficiency.Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesisPiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomesActivating transcription factor 6 limits intracellular accumulation of mutant α(1)-antitrypsin Z and mitochondrial damage in hepatoma cells.Linking of autophagy to ubiquitin-proteasome system is important for the regulation of endoplasmic reticulum stress and cell viability.Protein quality control: the who's who, the where's and therapeutic escapes.Oxidative stress contributes to liver damage in a murine model of alpha-1-antitrypsin deficiency.Sequestration of mutated alpha1-antitrypsin into inclusion bodies is a cell-protective mechanism to maintain endoplasmic reticulum function.Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy.Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency.The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB.Dissection of endoplasmic reticulum stress signaling in alcoholic and non-alcoholic liver injury.Disorders of protein misfolding: alpha-1-antitrypsin deficiency as prototype.Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulumFunctions of autophagy in normal and diseased liver.
P2860
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P2860
Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
2005年论文
@zh
2005年论文
@zh-cn
name
Accumulation of mutant alpha1- ...... the unfolded protein response.
@en
type
label
Accumulation of mutant alpha1- ...... the unfolded protein response.
@en
prefLabel
Accumulation of mutant alpha1- ...... the unfolded protein response.
@en
P356
P1476
Accumulation of mutant alpha1- ...... the unfolded protein response
@en
P2093
David H Perlmutter
Pamela Hale
P304
39002-39015
P356
10.1074/JBC.M508652200
P407
P577
2005-09-23T00:00:00Z